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Supplemental Table and Figure Legends Table S1. Clinical characteristics and hepatic enzyme activities of human participants. (-) NAFLD (N=9) (+) NAFLD (N=12) MALE (N=7) FEMALE (N=2) MALE (N=5) FEMALE (N=7) Age (years) 62±7.4 44±0 67±8.6 62±9.3 BMI (kg/m 2 ) 22.2±1.8 22.0±2.1 26.7±5.5 * 24.7±1.4 ## Comorbidities Hypertension (%) 42.9 50 60 42.9 Diabetes mellitus (%) 14.3 0 0 28.6 Dyslipidemia (%) 0 0 40 71.4 Liver biochemistries ALT(U/L) 17.7±8.1 9±0 18.6±13.3 18.7±10.1 AST(U/L) 20.3±8.2 11±2.8 22.2±6.8 18.3±3.8 Values expressed are mean ±SD. BMI, body mass index; AST, aspartate aminotransferase; ALT, alanine aminotransferase. *P<0.01, ## P<0.005 vs. controls Table S2. Identification of proteins associated with LDs of human liver sample 1. Reference MW Accession Scan(s) Sp RSp 1 clathrin heavy chain 1 191612.6 4758012 2 alcohol dehydrogenase 4 40221.5 71565152 3 long-chain-fatty-acid--CoA ligase 1 77942.9 40807491 4 ras GTPase-activating-like protein IQGAP2 180577.0 116089337 5 spectrin alpha chain, brain isoform 1 285091.3 194595509 6 dimethylaniline monooxygenase [N-oxide-forming] 3 60033.2 50541961 7 myosin-9 226529.8 12667788 8 tubulin beta-2A chain 49906.7 4507729 9 epoxide hydrolase 1 precursor 52948.5 209862837 10 bifunctional ATP-dependent dihydroxyacetone kinase/FAD-AMP lyase (cyclizing) 58946.8 20149621 11 17-beta-hydroxysteroid dehydrogenase 13 isoform B 29604.7 210032112 12 spectrin beta chain, brain 1 isoform 1 274606.5 112382250 13 peroxisomal multifunctional enzyme type 2 isoform 1 83024.9 313482810 14 alpha-actinin-4 104853.3 12025678
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Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

Dec 30, 2020

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Page 1: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

Supplemental Table and Figure Legends

Table S1. Clinical characteristics and hepatic enzyme activities of human participants.

(-) NAFLD (N=9) (+) NAFLD (N=12)

MALE (N=7)

FEMALE

(N=2)

MALE

(N=5)

FEMALE

(N=7)

Age (years) 62±7.4 44±0 67±8.6 62±9.3

BMI (kg/m2) 22.2±1.8 22.0±2.1 26.7±5.5

* 24.7±1.4

##

Comorbidities

Hypertension (%) 42.9 50 60 42.9

Diabetes mellitus (%) 14.3 0 0 28.6

Dyslipidemia (%) 0 0 40 71.4

Liver biochemistries

ALT(U/L) 17.7±8.1 9±0 18.6±13.3 18.7±10.1

AST(U/L) 20.3±8.2 11±2.8 22.2±6.8 18.3±3.8

Values expressed are mean ±SD.

BMI, body mass index; AST, aspartate aminotransferase; ALT, alanine aminotransferase.

*P<0.01, ##

P<0.005 vs. controls

Table S2. Identification of proteins associated with LDs of human liver sample 1.

Reference MW Accession

Scan(s) Sp RSp

1 clathrin heavy chain 1 191612.6 4758012

2 alcohol dehydrogenase 4 40221.5 71565152

3 long-chain-fatty-acid--CoA ligase 1 77942.9 40807491

4 ras GTPase-activating-like protein IQGAP2 180577.0 116089337

5 spectrin alpha chain, brain isoform 1 285091.3 194595509

6 dimethylaniline monooxygenase [N-oxide-forming] 3 60033.2 50541961

7 myosin-9 226529.8 12667788

8 tubulin beta-2A chain 49906.7 4507729

9 epoxide hydrolase 1 precursor 52948.5 209862837

10 bifunctional ATP-dependent dihydroxyacetone kinase/FAD-AMP lyase

(cyclizing) 58946.8 20149621

11 17-beta-hydroxysteroid dehydrogenase 13 isoform B 29604.7 210032112

12 spectrin beta chain, brain 1 isoform 1 274606.5 112382250

13 peroxisomal multifunctional enzyme type 2 isoform 1 83024.9 313482810

14 alpha-actinin-4 104853.3 12025678

Page 2: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

15 plectin isoform 1e 513705.9 41322908

16 methyltransferase-like protein 7A precursor 28318.9 89145417

17 UTP--glucose-1-phosphate uridylyltransferase isoform a 56939.9 48255966

18 apolipoprotein B-100 precursor 515528.6 105990532

19 fatty acid synthase 273423.2 41872631

20 ATP synthase subunit beta, mitochondrial precursor 56559.5 32189394

21 perilipin-2 48075.1 34577059

22 peroxisomal bifunctional enzyme isoform 2 69153.5 261878539

23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581

24 probable saccharopine dehydrogenase 47151.1 55770836

25 long-chain-fatty-acid--CoA ligase 5 isoform a 82262.5 42794756

26 estradiol 17-beta-dehydrogenase 11 32935.6 142976729

27 carbamoyl-phosphate synthase [ammonia], mitochondrial isoform c 116037.4 170295797

28 annexin A6 isoform 1 75872.8 71773329

29 tubulin alpha-1A chain 50135.3 17986283

30 trifunctional enzyme subunit alpha, mitochondrial precursor 82999.1 20127408

31 programmed cell death 6-interacting protein isoform 1 96022.6 22027538

32 17-beta-hydroxysteroid dehydrogenase type 6 35965.5 19743808

33 ATP synthase subunit alpha, mitochondrial precursor 59750.3 4757810

34 bile acyl-CoA synthetase precursor 75384.7 13325057

35 cytochrome P450 2E1 precursor 56848.6 10834998

36 formimidoyltransferase-cyclodeaminase 58926.2 11140815

37 NADH-cytochrome b5 reductase 3 isoform 2 31628.5 193794826

38 very long-chain acyl-CoA synthetase isoform 1 70311.8 227499619

39 D-3-phosphoglycerate dehydrogenase 56650.2 23308577

40 microsomal glutathione S-transferase 1 17598.6 9945306

41 prelamin-A/C isoform 1 precursor 74139.0 27436946

42 glutamate dehydrogenase 2, mitochondrial precursor 61433.6 31377775

43 acetyl-CoA carboxylase 2 precursor 276537.6 134142062

44 UDP-glucuronosyltransferase 2B4 precursor 60512.1 149944509

45 dehydrogenase/reductase SDR family member 1 33908.8 209862901

46 D-beta-hydroxybutyrate dehydrogenase, mitochondrial precursor 38156.9 44680133

47 UDP-glucuronosyltransferase 2B7 precursor 60720.3 190194389

48 villin-1 92694.4 194394237

49 17-beta-hydroxysteroid dehydrogenase 13 isoform A 33655.2 210032110

50 non-specific lipid-transfer protein isoform 1 proprotein 58993.3 19923233

51 cytochrome P450 2C9 precursor 55627.6 13699818

52 eukaryotic initiation factor 4A-II 46402.0 83700235

53 cytochrome P450 2C19 precursor 55944.8 4503219

54 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1

precursor 68569.0 4506675

55 protein transport protein Sec23A 86160.3 38202214

56 perilipin-3 isoform 1 47074.7 255958282

57 perilipin-1 55990.0 223718203

58 aldehyde dehydrogenase family 8 member A1 isoform 3 48035.6 301500698

59 liver carboxylesterase 1 isoform c precursor 62392.5 68508957

60 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial 72691.1 156416003

Page 3: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

61 tubulin beta chain 49670.5 29788785

62 elongation factor 2 95337.6 4503483

63 talin-1 269764.4 223029410

64 78 kDa glucose-regulated protein precursor 72332.5 16507237

65 serum paraoxonase/lactonase 3 39607.2 29788996

66 cytochrome P450 2D6 isoform 1 55730.1 40805836

67 amine oxidase [flavin-containing] B 58762.6 38202207

68 calnexin precursor 67567.8 10716563

69 vimentin 53651.3 62414289

70 corticosteroid 11-beta-dehydrogenase isozyme 1 32400.8 5031765

71 SEC14-like protein 2 isoform 1 46145.0 7110715

72 histone H4 11367.3 4504301

73 NADPH--cytochrome P450 reductase 77047.7 127139033

74 synaptic vesicle membrane protein VAT-1 homolog 41920.0 18379349

75 coatomer subunit alpha isoform 2 138344.7 148536853

76 lanosterol synthase isoform 2 82198.1 224177556

77 ADP/ATP translocase 2 32852.0 156071459

78 cullin-associated NEDD8-dissociated protein 1 136374.7 21361794

79 cytochrome P450 3A5 isoform 1 57108.3 4503231

80 actin, gamma-enteric smooth muscle isoform 1 precursor 41876.7 4501889

81 ras-related protein Rab-7a 23489.6 34147513

82 voltage-dependent anion-selective channel protein 3 isoform 2 30789.6 208879465

83 perilipin-4 134430.4 122937195

84 endoplasmin precursor 92468.2 4507677

85 alcohol dehydrogenase 1B 39835.3 34577061

86 protein ERGIC-53 precursor 57548.6 5031873

87 actin, cytoplasmic 1 41736.5 4501885

88 bile salt sulfotransferase 33779.6 29540545

89 sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating 41900.0 8393516

90 myosin-Ib isoform 2 124949.7 44889481

91 aldehyde dehydrogenase, mitochondrial isoform 1 precursor 56380.9 25777732

92 membrane-associated progesterone receptor component 1 21671.0 5729875

93 AP-2 complex subunit alpha-1 isoform 2 105360.8 19913416

94 C-1-tetrahydrofolate synthase, cytoplasmic 101530.5 222136639

95 cathepsin D preproprotein 44552.0 4503143

96 hemoglobin subunit beta 15998.3 4504349

97 myosin-10 228996.6 41406064

98 AP-2 complex subunit beta isoform b 104551.9 4557469

99 desmoplakin isoform I 331771.4 58530840

100 hemoglobin subunit delta 16055.4 4504351

101 cytochrome b5 isoform 1 15330.0 41281768

102 ras-related protein Rab-14 23896.8 19923483

103 60S ribosomal protein L4 47697.0 16579885

104 hemoglobin subunit alpha 15257.4 4504345

105 serum paraoxonase/arylesterase 1 precursor 39731.0 19923106

106 fatty aldehyde dehydrogenase isoform 1 57669.1 73466520

107 7-alpha-hydroxycholest-4-en-3-one 12-alpha-hydroxylase 58068.1 166362724

108 cytochrome P450 3A7 57525.2 262290932

Page 4: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

109 trifunctional enzyme subunit beta, mitochondrial precursor 51294.1 4504327

110 apoptosis-inducing factor 1, mitochondrial isoform 2 precursor 66294.3 22202629

111 delta(24)-sterol reductase precursor 60100.9 13375618

112 annexin A4 36084.8 4502105

113 coatomer subunit beta 107141.5 7705369

114 cytochrome P450 2C18 isoform 1 precursor 55710.4 13699816

115 tricarboxylate transport protein, mitochondrial precursor 34012.4 21389315

116 histone H2B type 1-C/E/F/G/I 13906.0 4504271

117 NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform 3 67523.9 316983158

118 protein diaphanous homolog 1 isoform 1 141346.4 119395758

119 protein transport protein Sec24A isoform 1 119748.6 116174780

120 cytochrome P450 4F11 precursor 60145.5 193083178

121 coatomer subunit gamma 97717.7 11559929

122 carbamoyl-phosphate synthase [ammonia], mitochondrial isoform a precursor 165648.8 169790915

123 very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2

precursor 68058.1 76496475

124 calcium-binding mitochondrial carrier protein Aralar1 74761.4 21361103

125 amine oxidase [flavin-containing] A 59681.3 4557735

126 peroxisomal multifunctional enzyme type 2 isoform 2 79685.9 4504505

127 fructose-bisphosphate aldolase B 39472.8 40354205

128 protein disulfide-isomerase precursor 57115.9 20070125

129 dimethylaniline monooxygenase [N-oxide-forming] 5 isoform 1 60220.2 221316672

130 estradiol 17-beta-dehydrogenase 2 42784.9 4504503

131 nicotinamide N-methyltransferase 29573.8 5453790

132 UDP-glucuronosyltransferase 1-7 precursor 59818.5 41282213

133 mitochondrial carrier homolog 2 33330.7 7657347

134 surfeit locus protein 4 30393.8 19557691

135 epoxide hydrolase 2 62615.4 27597073

136 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2

isoform 1 precursor 69283.5 35493916

137 heat shock protein HSP 90-alpha isoform 1 98160.4 153792590

138 heat shock protein HSP 90-beta 83263.6 20149594

139 glutamate dehydrogenase 1, mitochondrial precursor 61397.5 4885281

140 retinol dehydrogenase 10 38087.3 25282469

141 prohibitin-2 isoform 1 33296.1 221307584

142 NAD(P) transhydrogenase, mitochondrial precursor 113894.8 122939155

143 oxysterol-binding protein 1 89420.0 4505531

144 vesicle-trafficking protein SEC22b precursor 24593.2 94429050

145 cytochrome P450 3A4 isoform 2 57255.8 322302351

146 catenin alpha-1 100070.6 55770844

147 signal transducer and activator of transcription 3 isoform 1 88067.3 21618340

148 phosphoglucomutase-1 isoform 1 61448.7 21361621

149 serine--pyruvate aminotransferase 43009.7 4557289

150 mitochondrial carnitine/acylcarnitine carrier protein 32943.5 4557403

151 adipocyte plasma membrane-associated protein 46480.1 24308201

152 translational activator GCN1 292707.0 54607053

Page 5: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

153 7-dehydrocholesterol reductase 54489.1 119943112

154 reticulon-4 isoform A 129930.3 24431935

155 sorbitol dehydrogenase 38324.3 156627571

156 ATP-binding cassette sub-family D member 3 isoform a 75475.5 4506341

157 cytoplasmic dynein 1 heavy chain 1 532406.6 33350932

158 60S ribosomal protein L3 isoform a 46108.6 4506649

159 voltage-dependent anion-selective channel protein 1 30772.3 4507879

160 mitochondrial dicarboxylate carrier 31282.3 20149598

161 myosin-Ia 118399.8 4885503

162 transmembrane emp24 domain-containing protein 9 precursor 27277.2 39725636

163 C4b-binding protein alpha chain precursor 67032.9 4502503

164 serum albumin preproprotein 69366.4 4502027

165 dynamin-1-like protein isoform 1 81876.7 171460914

166 40S ribosomal protein S8 24205.0 4506743

167 vesicle-associated membrane protein-associated protein A isoform 1 32613.3 94721250

168 monoglyceride lipase isoform 1 34292.3 6005786

169 abhydrolase domain-containing protein 14B 22345.5 14249382

170 40S ribosomal protein SA 32853.8 59859885

171 ras-related protein Rab-2A isoform a 23545.4 4506365

172 sodium/potassium-transporting ATPase subunit alpha-1 isoform c 112999.5 237681109

173 poly(rC)-binding protein 1 37497.6 222352151

174 tripeptidyl-peptidase 1 preproprotein 61247.6 5729770

175 elongation factor 1-alpha 2 50469.8 4503475

176 protein disulfide-isomerase A4 precursor 72931.9 4758304

177 PREDICTED: POTE ankyrin domain family member I isoform 2 121281.5 88953571

178 protein transport protein Sec31A isoform 1 133013.8 116256338

179 filamin-B isoform 2 278160.3 105990514

180 cytochrome P450 2A7 isoform 2 precursor 50666.4 15147328

181 lipopolysaccharide-responsive and beige-like anchor protein isoform 2 319105.2 148233596

182 stromal interaction molecule 1 precursor 77422.9 21070997

183 glycerate kinase isoform 1 55252.3 31543063

184 calpain-1 catalytic subunit 81889.4 12408656

185 serotransferrin precursor 77049.5 4557871

186 hydroxymethylglutaryl-CoA synthase, mitochondrial isoform 1 precursor 56635.1 5031751

187 histone H2A type 1-D 14107.4 10800130

188 retinol dehydrogenase 16 35673.2 150247226

189 L-xylulose reductase isoform 2 25742.7 304571975

190 tubulin beta-8 chain isoform 1 49775.7 42558279

191 cytochrome P450 2C8 isoform b 47785.8 311893309

192 short-chain dehydrogenase/reductase 3 33548.2 31543615

193 UDP-glucuronosyltransferase 2A2 60771.7 333609245

194 ribosome-binding protein 1 108654.8 110611220

195 myosin-11 isoform SM2A 223574.8 13124875

196 junction plakoglobin 81744.3 12056468

197 myosin-14 isoform 3 232008.3 224831241

198 UDP-glucuronosyltransferase 2B28 isoform 2 precursor 38743.0 333033811

199 cytochrome P450 2J2 57610.3 18491008

200 glycine N-acyltransferase isoform a 33924.0 111038137

Page 6: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

201 bromodomain adjacent to zinc finger domain protein 2B 240456.7 94681063

202 cytochrome P450 4A11 59347.5 158937242

203 sepiapterin reductase 28048.3 4507185

204 electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial

precursor 68495.0 119703746

205 tubulin beta-2C chain 49830.7 5174735

206 glutathione S-transferase kappa 1 isoform a 25496.7 7705704

207 phosphatidylinositol-binding clathrin assembly protein isoform 1 70754.1 56788366

208 SEC14-like protein 3 46047.9 27923592

209 UDP-glucuronosyltransferase 1-4 precursor 60024.7 6005930

210 sulfotransferase 1A2 34310.4 4507303

211 vigilin isoform b 137969.8 345199283

212 pyruvate kinase isozymes R/L isoform 1 61829.8 10835121

213 OCIA domain-containing protein 2 isoform 1 16953.4 62244044

214 heterogeneous nuclear ribonucleoprotein H2 49263.3 9624998

215 kynurenine 3-monooxygenase 55809.7 187960045

216 fermitin family homolog 2 isoform 3 72396.9 201861823

217 UDP-glucuronosyltransferase 2B15 precursor 61036.0 116517299

218 tubulin alpha-1B chain 50151.3 57013276

219 aldehyde dehydrogenase family 8 member A1 isoform 1 53401.1 12007648

220 transmembrane protein 33 27978.0 224589127

221 ras-related protein Rap-1b isoform 2 16033.3 354459350

222 elongation factor 1-alpha 1 50140.5 4503471

223 ceramide synthase 2 44875.8 31077094

224 cytochrome c oxidase subunit II 25564.8 251831110

225 epiplakin 555623.1 207452735

226 phosphatidate cytidylyltransferase 2 51417.7 20143480

227 uridine 5'-monophosphate synthase 52221.3 4507835

228 arginase-1 isoform 2 34734.7 10947139

229 clathrin interactor 1 isoform 1 70294.4 307078123

230 coatomer subunit gamma-2 97621.8 109134349

231 microsomal glutathione S-transferase 3 16516.2 4758714

232 peroxisomal membrane protein 2 22252.4 8923892

233 hydroxyacid oxidase 1 40924.1 11068137

234 transmembrane protein 205 21197.7 63055043

235 UDP-glucuronosyltransferase 1-9 precursor 59940.7 11276085

236 guanidinoacetate N-methyltransferase isoform a 26318.0 4503909

237 estradiol 17-beta-dehydrogenase 12 34323.9 7705855

238 staphylococcal nuclease domain-containing protein 1 101996.3 77404397

239 UDP-glucuronosyltransferase 2B10 isoform 2 precursor 50688.0 221219059

240 POTE ankyrin domain family member E 121362.7 134133226

241 transmembrane emp24 domain-containing protein 10 precursor 24975.8 98986464

242 POTE ankyrin domain family member F 121443.8 153791352

243 protein disulfide-isomerase A6 precursor 48121.0 5031973

244 mitochondrial import receptor subunit TOM70 67454.4 54607135

245 malectin precursor 32233.6 7661948

246 phosphate carrier protein, mitochondrial isoform b precursor 39958.5 47132595

247 importin subunit beta-1 97169.7 19923142

Page 7: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

248 core histone macro-H2A.1 isoform 1 39183.2 20336746

249 prohibitin 29803.9 4505773

250 dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit

precursor 50701.3 20070197

251 serum paraoxonase/arylesterase 2 isoform 1 39380.6 66529294

252 nuclear pore membrane glycoprotein 210 precursor 205108.8 27477134

253 transitional endoplasmic reticulum ATPase 89321.3 6005942

254 methyltransferase-like protein 7B precursor 27774.6 164663805

255 aflatoxin B1 aldehyde reductase member 3 37206.2 41152114

256 regulator of microtubule dynamics protein 2 isoform 2 47399.1 283046674

257 catenin beta-1 85496.2 4503131

258 erlin-2 isoform 1 37839.3 6005721

259 60S ribosomal protein L8 28024.4 15431306

260 guanine nucleotide-binding protein subunit beta-2-like 1 35076.5 5174447

261 transmembrane emp24 domain-containing protein 2 precursor 22761.1 5803149

262 beta-soluble NSF attachment protein 33556.8 44917606

263 GTP-binding protein SAR1a 22366.6 217416369

264 cadherin-1 preproprotein 97455.4 4757960

265 glycerol-3-phosphate acyltransferase 1, mitochondrial precursor 93794.2 190358539

266 histone H2B type 1-O 13906.0 16306566

267 heat shock-related 70 kDa protein 2 70020.5 13676857

268 peripherin 53650.5 21264345

269 mitochondrial inner membrane protein isoform 1 83677.3 154354964

270 mitochondrial glutamate carrier 1 34470.0 13375983

271 microsomal triglyceride transfer protein large subunit precursor 99350.6 153285408

272 40S ribosomal protein S3a 29944.7 4506723

273 F-actin-capping protein subunit beta isoform 2 31350.3 330864679

274 lysophospholipid acyltransferase 5 56034.7 42542394

275 tyrosine aminotransferase 50399.2 4507369

276 ras-related protein Rab-1A isoform 1 22677.6 4758988

277 clarin-3 25321.2 22748685

278 enoyl-CoA hydratase domain-containing protein 1 isoform 1 32997.0 157694516

279 glutathione S-transferase A3 25301.5 24430144

280 eukaryotic translation initiation factor 3 subunit L isoform 1 66726.6 7705433

281 26S protease regulatory subunit 10B 45796.7 195539395

282 cytochrome P450 1A2 58407.1 73915100

283 ras-related protein Rab-2B isoform 1 24214.2 21361884

284 dual specificity mitogen-activated protein kinase kinase 4 44287.5 4506889

285 galactokinase 42272.0 4503895

286 hypoxia up-regulated protein 1 precursor 111334.8 5453832

287 PREDICTED: liver carboxylesterase 1-like 27540.5 341916335

288 perilipin-5 50791.1 116292172

289 signal recognition particle receptor subunit beta 29702.0 284795266

290 argininosuccinate synthase 46530.1 16950633

291 plastin-3 isoform 1 70810.6 7549809

292 LETM1 domain-containing protein 1 isoform 1 41790.1 67089165

293 extended synaptotagmin-1 isoform 2 122855.6 14149680

294 guanine nucleotide-binding protein G(I)/G(S)/G(T) subunit beta-2 37330.8 20357529

Page 8: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

295 EH domain-containing protein 3 60886.7 7657056

296 PREDICTED: complement C4-A isoform 1 192843.7 341916194

297 exportin-2 110415.8 29029559

298 ribonuclease inhibitor 49973.3 42822872

299 myosin-Ic isoform c 117906.3 124494240

300 malate dehydrogenase, cytoplasmic isoform 2 36425.9 5174539

301 retinal dehydrogenase 1 54861.5 21361176

302 general vesicular transport factor p115 107906.7 4505541

303 ranBP2-like and GRIP domain-containing protein 4 197286.6 211059431

304 DCC-interacting protein 13-alpha 79663.1 6912242

305 probable ATP-dependent RNA helicase DDX17 isoform 1 80272.0 38201710

306 coatomer subunit delta isoform 1 57210.1 11863154

307 DNA damage-binding protein 1 126967.0 148529014

308 alpha-actinin-1 isoform b 103056.9 4501891

309 EH domain-containing protein 1 60626.4 30240932

310 apolipoprotein E precursor 36153.8 4557325

311 scavenger receptor cysteine-rich type 1 protein M130 isoform a precursor 125450.2 344179110

312 alpha-tocopherol transfer protein-like 38514.9 85861250

313 exocyst complex component 5 81852.4 5730037

314 torsin-1A-interacting protein 1 66248.0 39753957

315 solute carrier organic anion transporter family member 3A1 isoform 2 74372.1 222831573

316 long-chain-fatty-acid--CoA ligase 3 80419.6 42794752

317 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9,

mitochondrial precursor 42509.3 6681764

318 apolipoprotein L2 37092.1 13562090

319 protein DJ-1 19890.9 31543380

320 alkylglycerol monooxygenase 51499.6 51972212

321 heterogeneous nuclear ribonucleoprotein K isoform a 51027.9 14165437

322 transmembrane protein 109 precursor 26209.8 13129092

323 erlin-1 39170.9 154800487

324 OCIA domain-containing protein 1 isoform 1 27626.0 8923427

325 lysosome-associated membrane glycoprotein 1 precursor 44882.1 112380628

326 60S ribosomal protein L18 21634.3 4506607

327 MOSC domain-containing protein 2, mitochondrial precursor 38023.2 31542713

328 ras-related protein Rab-10 22540.8 256222019

329 alcohol dehydrogenase [NADP+] 36572.8 24497577

330 calreticulin precursor 48141.1 4757900

331 enoyl-CoA hydratase, mitochondrial precursor 31387.2 194097323

332 B-cell CLL/lymphoma 9 protein 149289.3 72256200

333 ADP-ribosylation factor GTPase-activating protein 2 isoform 1 56720.0 31543983

334 long-chain-fatty-acid--CoA ligase 6 isoform d 77670.3 327412323

335 cytochrome P450 2A13 56687.2 13699809

336 ras GTPase-activating-like protein IQGAP1 189250.1 4506787

337 splicing factor, proline- and glutamine-rich 76148.9 4826998

338 probable G-protein coupled receptor 110 isoform 1 precursor 101363.9 61743940

339 sorting nexin-29 91253.1 343780938

340 3-keto-steroid reductase 38205.9 7705421

341 3-hydroxyacyl-CoA dehydratase 3 43159.3 117168248

Page 9: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

342 myomegalin isoform 8 254042.9 311771516

343 eukaryotic peptide chain release factor GTP-binding subunit ERF3A isoform 1 68671.3 194018520

344 signal recognition particle receptor subunit alpha isoform 1 69810.8 23308697

345 protocadherin-10 isoform 1 precursor 112935.2 14589916

346 fatty acid-binding protein, liver 14208.3 4557577

347 peroxisomal trans-2-enoyl-CoA reductase 32544.1 19923817

348 prenylcysteine oxidase 1 precursor 56639.8 166795301

349 NEDD4-binding protein 1 100378.1 48928019

350 AP-1 complex subunit gamma-1 isoform b 91351.0 71772942

351 homeobox protein MIXL1 24658.8 13994335

352 peroxiredoxin-1 22110.2 4505591

353 5'-AMP-activated protein kinase subunit beta-2 30302.0 4885561

354 UDP-glucuronosyltransferase 2B11 precursor 61037.9 4507823

355 endoplasmic reticulum-Golgi intermediate compartment protein 1 32592.1 72534712

356 terminal uridylyltransferase 7 isoform 2 144655.2 297374764

357 glyceraldehyde-3-phosphate dehydrogenase 36053.0 7669492

358 heterogeneous nuclear ribonucleoprotein M isoform a 77515.2 14141152

359 membrane-associated progesterone receptor component 2 26169.9 291621647

360 3-mercaptopyruvate sulfurtransferase isoform 2 33178.2 61835204

361 ER lumen protein retaining receptor 2 isoform 1 24421.7 5803050

362 rho-related GTP-binding protein RhoB precursor 22123.3 4757764

363 X-ray repair cross-complementing protein 5 82704.0 10863945

364 serine/threonine-protein kinase OSR1 58021.8 4826878

365 enoyl-CoA delta isomerase 2, mitochondrial isoform 1 40182.8 45643119

366 glutathione S-transferase A5 25721.8 24308514

367 redox-regulatory protein PAMM isoform 1 precursor 25764.0 344925828

368 TRAM adaptor with GOLD domain isoform 2 precursor 21232.8 256985102

369 reticulon-4 isoform C 22395.3 5902016

370 protein disulfide-isomerase A3 precursor 56782.0 21361657

371 serpin H1 precursor 46440.3 333360851

372 AP-2 complex subunit alpha-2 isoform 1 104088.9 338827685

373 beta-2-glycoprotein 1 precursor 38298.0 153266841

374 cytoskeleton-associated protein 4 66022.0 19920317

375 bcl-2-like protein 13 52722.8 45243501

376 transmembrane emp24 domain-containing protein 5 isoform 1 precursor 26004.7 282165814

377 glutamate receptor 1 isoform 1 precursor 101505.4 167001419

378 serine/threonine-protein phosphatase 6 regulatory subunit 2 isoform 4 101006.2 339276044

379 protein kinase C alpha type 76763.6 4506067

380 cytochrome P450 2A6 precursor 56517.1 189339233

381 hemoglobin subunit epsilon 16202.7 4885393

382 leukotriene-B(4) omega-hydroxylase 2 isoform a 59846.4 119220562

383 aspartyl/asparaginyl beta-hydroxylase isoform f 83267.4 258613943

384 coiled-coil domain-containing protein 108 isoform 1 217247.4 83035129

385 ras-related protein Rab-18 22976.9 10880989

386 dyslexia-associated protein KIAA0319-like protein 115656.9 33359221

387 actin-related protein 2 isoform a 45376.2 53692187

388 apolipoprotein C-I precursor 9331.9 4502157

Page 10: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

389 RING finger protein 17 isoform 2 184211.1 297139728

390 dihydropyrimidinase-related protein 4 61877.3 190194363

391 derlin-1 isoform b 26422.7 197927278

392 probable ATP-dependent RNA helicase DDX6 54416.5 164664518

393 60S ribosomal protein L13a 23577.1 6912634

394 ATP synthase subunit gamma, mitochondrial isoform H (heart) precursor 32880.7 4885079

395 DNA mismatch repair protein Mlh3 isoform 2 161019.7 91992160

396 uncharacterized protein C2orf47, mitochondrial precursor 32544.4 239582772

397 beta-actin-like protein 2 42002.9 63055057

398 succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial

precursor 31629.6 115387094

399 dehydrogenase/reductase SDR family member 7B 35118.8 20149619

400 arylacetamide deacetylase 45733.4 68299767

401 WD repeat-containing protein 62 isoform 2 165952.0 145580610

402 40S ribosomal protein S25 13742.0 4506707

403 obg-like ATPase 1 isoform 1 44743.2 58761500

404 general receptor for phosphoinositides 1-associated scaffold protein 42623.1 32171221

405 HLA class I histocompatibility antigen, A-1 alpha chain precursor 40840.5 24797067

406 peroxisomal bifunctional enzyme isoform 1 79494.4 68989263

407 leukotriene-B(4) omega-hydroxylase 1 precursor 59853.2 13435391

408 cytochrome P450 4A22 59245.5 62952506

409 peroxisomal membrane protein 11B isoform 1 28431.1 4505719

410 nesprin-3 112215.8 145580592

411 lysosome-associated membrane glycoprotein 2 isoform A precursor 44960.5 4504957

412 ras-related protein Rab-5A 23658.5 19923262

413 protein NipSnap homolog 1 isoform 1 33309.7 193211616

414 Down syndrome critical region protein 3 33010.2 5174425

415 acid ceramidase isoform b 46503.3 189011546

416 vitronectin precursor 54305.3 88853069

417 solute carrier family 2, facilitated glucose transporter member 2 57489.2 4557851

418 membrane primary amine oxidase 84621.4 4502119

419 phosphatidylinositol 4-kinase type 2-alpha 54022.0 13559514

420 60S acidic ribosomal protein P1 isoform 1 11513.9 4506669

421 3-ketoacyl-CoA thiolase, peroxisomal isoform a 44291.8 4501853

422 serine hydroxymethyltransferase, cytosolic isoform 1 53082.3 22547186

423 protein PRRC2A 228860.0 149158690

424 T-complex protein 1 subunit delta 57924.0 38455427

425 60S ribosomal protein L7 29225.6 15431301

426 ADP/ATP translocase 1 33064.2 55749577

427 uncharacterized protein C2orf72 30480.5 222418604

428 cyclin-dependent kinase inhibitor 3 isoform 2 19359.1 195927025

429 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform 2 65522.6 94557299

430 FAS-associated factor 2 52623.1 24797106

431 fatty acid-binding protein, intestinal 15237.2 194097325

432 alcohol dehydrogenase 1C 39867.4 4501933

433 chromodomain-helicase-DNA-binding protein 8 isoform 2 262344.5 114326455

434 protein transport protein Sec24B isoform a 137416.6 112382212

435 myosin-VI 148712.8 92859701

Page 11: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

436 E3 ubiquitin-protein ligase TRIM23 isoform alpha 64066.3 4502197

437 zinc finger CCHC domain-containing protein 3 43547.1 29648305

438 guanine nucleotide-binding protein G(i) subunit alpha-2 isoform 1 40450.7 4504041

439 importin subunit alpha-3 57810.5 34485722

440 N-acetyl-beta-glucosaminyl-glycoprotein 4-beta-N-

acetylgalactosaminyltransferase 2 114974.2 71043500

441 gamma-aminobutyric acid receptor subunit beta-2 isoform 1 precursor 59150.1 12548785

442 ras-related protein Rab-1B 22171.0 13569962

443 transmembrane emp24 domain-containing protein 4 precursor 25942.6 33457308

444 ephrin type-A receptor 2 precursor 108265.8 32967311

445 protein OSCP1 isoform 1 43270.6 268370167

446 exportin-7 123906.4 154448892

447 ATP-dependent DNA helicase Q4 133076.5 284005309

448 leiomodin-2 61675.0 150378503

449 structural maintenance of chromosomes protein 2 135655.0 110347420

450 peroxisomal membrane protein PEX14 41236.3 4758896

451 uncharacterized protein C12orf56 isoform 2 53561.4 153791218

452 endothelial lipase precursor 56794.3 5174497

453 6-phosphofructokinase type C isoform 2 85315.5 334191701

454 rho guanine nucleotide exchange factor 2 isoform 1 111542.1 253735775

455 RANBP2-like and GRIP domain-containing protein 1/2 196659.8 262118265

456 zinc finger protein 644 isoform 2 11723.8 41152091

457 isochorismatase domain-containing protein 1 32236.5 103471987

458 X-linked interleukin-1 receptor accessory protein-like 2 precursor 78669.4 11225607

459 exportin-5 136310.3 22748937

460 nucleoprotein TPR 267289.5 114155142

461 cadherin-4 isoform 2 96396.6 356640221

462 charged multivesicular body protein 5 isoform 1 24570.6 189409150

463 heat shock 70 kDa protein 1A/1B 70051.8 194248072

464 DNA repair protein RAD52 homolog 46168.4 109637798

465 BTB/POZ domain-containing protein 18 77931.0 223029436

466 calmodulin-regulated spectrin-associated protein 2 166730.9 44955929

467 protein Muted homolog isoform 1 21609.4 41152237

468 neurolysin, mitochondrial precursor 80651.4 14149738

469 myotubularin-related protein 2 isoform 1 73380.8 44680154

470 MIT domain-containing protein 1 29314.2 20270349

471 proline-rich AKT1 substrate 1 27383.1 148806896

472 splicing factor 3B subunit 1 isoform 1 145829.1 54112117

473 magnesium transporter protein 1 41531.6 215983058

474 dihydropyrimidine dehydrogenase [NADP+] isoform 1 111400.8 119943098

475 cingulin 137055.9 16262452

476 LIM homeobox transcription factor 1-beta isoform 1 44101.8 292494911

477 guanylate cyclase soluble subunit alpha-3 isoform A 77452.1 67763816

478 T-complex protein 1 subunit theta 59620.3 48762932

479 zinc finger protein 878 61539.7 332634996

480 pleckstrin homology domain-containing family G member 2 147968.0 164565408

481 interferon regulatory factor 5 isoform b 56043.8 14249182

482 protein MMS22-like 142319.4 115583683

Page 12: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

483 numb-like protein 64891.1 10863899

484 epsin-2 isoform b 68481.2 156671215

485 nuclear pore complex protein Nup205 227917.8 57634534

486 microtubule-actin cross-linking factor 1 isoform a 620419.4 33188445

487 thrombospondin-1 precursor 129381.9 40317626

488 dachshund homolog 2 isoform a 65322.5 16876441

489 dynein heavy chain 10, axonemal 514840.2 198442844

490 chloride intracellular channel protein 5 isoform a 46502.3 166197662

491 protein furry homolog 338871.6 117606355

492 trifunctional purine biosynthetic protein adenosine-3 isoform 1 107766.5 209869993

493 probable ATP-dependent RNA helicase DDX60-like 197671.5 149274648

494 ras-related and estrogen-regulated growth inhibitor isoform 1 22607.7 14249704

495 syntaxin-binding protein 4 61661.4 63999048

496 kelch-like protein 6 70358.5 109150407

497 tumor necrosis factor alpha-induced protein 2 72660.9 26051240

498 pericentriolar material 1 protein 228530.8 134142826

499 polyamine-modulated factor 1-binding protein 1 isoform a 117493.1 237858619

500 WD repeat-containing protein 73 41684.8 116235476

501 PR domain zinc finger protein 2 isoform a 188913.7 20336258

502 ankyrin repeat domain-containing protein 31 210813.4 256574792

503 GTP-binding nuclear protein Ran 24422.9 5453555

504 probable E3 ubiquitin-protein ligase MID2 isoform 1 83209.8 223890257

505 aminopeptidase Q 113281.8 194239713

506 uncharacterized protein KIAA1107 149444.1 197245440

507 prestin isoform b 74843.3 45827800

508 sperm-associated antigen 17 251738.6 46240864

509 zinc finger protein 136 62783.4 4507987

510 astrotactin-1 isoform 2 precursor 135081.8 46488921

511 oligodendrocyte transcription factor 2 32384.4 17978475

512 RWD domain-containing protein 2A 33892.7 156523253

513 histone-lysine N-methyltransferase MLL3 541368.5 91718902

514 HLA class II histocompatibility antigen gamma chain isoform b 26398.5 10835071

515 cytochrome b 42717.3 251831119

516 U2 snRNP-associated SURP motif-containing protein 118291.2 122937227

517 kinesin heavy chain isoform 5C 109494.1 4758650

518 60S ribosomal protein L14 23431.7 78000181

519 glutamate-rich WD repeat-containing protein 1 49418.9 237820620

520 docking protein 3 isoform 2 35734.4 221554539

521 lipase maturation factor 2 79697.4 255918129

522 PREDICTED: hypothetical protein LOC100653074 21445.9 341913974

523 fibroblast growth factor-binding protein 2 precursor 24580.9 13994345

524 oxysterol-binding protein-related protein 8 isoform a 101194.8 18079218

525 SET-binding protein isoform a 175005.8 194294554

526 ryanodine receptor 1 isoform 1 565179.0 113204615

527 serine/threonine-protein kinase ATR 301363.9 157266317

528 biliverdin reductase A precursor 33428.3 33589854

529 chromodomain-helicase-DNA-binding protein 6 305409.5 29244924

530 uncharacterized protein C14orf45 61986.5 166295204

Page 13: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

531 muscle-related coiled-coil protein 41898.9 66472922

532 myosin-binding protein C, fast-type 128070.8 133908641

533 uncharacterized protein C10orf12 137222.6 24431975

534 heterogeneous nuclear ribonucleoprotein L isoform a 64132.4 52632383

535 geminin 23565.0 7705682

536 FERM, RhoGEF and pleckstrin domain-containing protein 2 119887.7 7662310

537 kinesin-like protein KIF24 151902.2 154426306

538 probable G-protein coupled receptor 113 isoform 2 precursor 107019.7 223556014

539 exocyst complex component 6 isoform b 93406.4 62243652

540 mediator of RNA polymerase II transcription subunit 1 168476.9 28559039

541 ubiquitin carboxyl-terminal hydrolase 35 113404.5 148746183

542 phosphorylase b kinase gamma catalytic chain, skeletal muscle isoform 45023.5 5453882

543 alpha-2-macroglobulin precursor 163290.5 66932947

544 matrix metalloproteinase-19 isoform rasi-1 preproprotein 57356.6 4505213

545 RNA-binding protein 42 50413.7 21359951

546 ATP-binding cassette sub-family A member 6 184284.2 27436953

547 leucine-rich repeat-containing protein 59 34930.2 40254924

548 putative tRNA pseudouridine synthase Pus10 60243.8 207113130

549 histone H3-like centromeric protein A isoform a 15990.4 4502775

550 MAGUK p55 subfamily member 5 77293.0 38570142

551 lysine-specific demethylase PHF2 120774.2 117190342

552 SH3 domain-containing protein 21 isoform 2 71237.7 242117948

553 uncharacterized protein KIAA1109 555478.9 150378498

554 methyltransferase-like protein 13 isoform 3 61127.3 55956895

555 transcription elongation regulator 1 isoform 1 123900.1 21327715

556 spatacsin isoform 1 278865.1 93204888

557 ubiquitin-like protein 4A 17776.4 7657667

558 DNA repair protein XRCC4 isoform 2 38286.5 12408647

559 SPS1/STE20-related protein kinase YSK4 isoform 2 57658.6 63998898

560 ras GTPase-activating protein nGAP isoform 1 128556.9 4758808

561 protein kinase C-binding protein 1 isoform b 128370.1 34335266

562 UPF0471 protein C1orf63 33613.1 46309849

563 oligodendrocyte transcription factor 3 29357.4 28411948

564 E3 ubiquitin-protein ligase RNF123 148513.5 37588869

565 bromodomain-containing protein 7 isoform 2 74138.1 41350212

566 uncharacterized protein C20orf177 isoform 1 42051.2 32698752

567 uncharacterized protein C9orf174 197342.3 302565871

568 growth arrest-specific protein 7 isoform b 47679.6 41406078

569 piezo-type mechanosensitive ion channel component 2 318060.9 257900451

570 ras-related protein Rab-8B 23584.0 7706563

571 copper-transporting ATPase 1 163372.3 115529486

572 disintegrin and metalloproteinase domain-containing protein 12 isoform 1

preproprotein 99541.7 73747885

573 kinesin-like protein KIF20A 100277.4 5032013

574 SHC SH2 domain-binding protein 1-like protein 72631.7 93204885

575 complement component C7 precursor 93517.9 45580688

576 DNA nucleotidylexotransferase isoform 1 58535.9 63054850

577 nephrocystin-3 150862.8 34304360

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578 coiled-coil domain-containing protein 80 precursor 108173.0 41152074

579 PDZ domain-containing protein 6 105647.5 44888833

580 filamin A-interacting protein 1-like isoform 1 130380.8 109659845

581 Golgin subfamily B member 1 376015.7 148596984

582 60S ribosomal protein L5 34362.4 14591909

583 sodium/hydrogen exchanger 9B1 isoform 1 56062.3 66793392

584 gamma-interferon-inducible protein 16 isoform 1 82587.7 330864753

585 G2/mitotic-specific cyclin-B3 isoform 3 157914.8 90669307

586 CD180 antigen precursor 74178.8 167555127

587 Golgi phosphoprotein 3 33810.3 11545859

588 Golgi SNAP receptor complex member 2 isoform B 24583.6 16905520

589 phosphatidylinositol-4-phosphate 5-kinase type-1 beta isoform 2 61036.0 4505817

590 collagen alpha-1(XX) chain 135829.1 112734845

591 calcineurin-binding protein cabin-1 isoform b 240762.4 319738622

592 apoptosis-stimulating of p53 protein 1 119564.7 121114287

593 monoacylglycerol lipase ABHD6 38330.4 189027141

594 uncharacterized protein C1orf141 46134.9 61966795

595 transmembrane protein 2 isoform a 154372.2 7019555

596 dnaJ homolog subfamily C member 16 precursor 90590.8 56687498

Table S3. Identification of proteins associated with LDs of human liver sample 2.

Reference MW Accession

Scan(s) Sp RSp

1 apolipoprotein B-100 precursor 515528.6 105990532

2 long-chain-fatty-acid--CoA ligase 1 77942.9 40807491

3 epoxide hydrolase 1 precursor 52948.5 209862837

4 fatty acid synthase 273423.2 41872631

5 alcohol dehydrogenase 4 40221.5 71565152

6 clathrin heavy chain 1 191612.6 4758012

7 cytochrome P450 2E1 precursor 56848.6 10834998

8 tubulin beta-2A chain 49906.7 4507729

9 bile acyl-CoA synthetase precursor 75384.7 13325057

10 17-beta-hydroxysteroid dehydrogenase 13 isoform B 29604.7 210032112

11 dehydrogenase/reductase SDR family member 1 33908.8 209862901

12 UTP--glucose-1-phosphate uridylyltransferase isoform a 56939.9 48255966

13 NADPH--cytochrome P450 reductase 77047.7 127139033

14 UDP-glucuronosyltransferase 2B7 precursor 60720.3 190194389

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15 ATP synthase subunit alpha, mitochondrial precursor 59750.3 4757810

16 lanosterol synthase isoform 2 82198.1 224177556

17 spectrin beta chain, brain 1 isoform 1 274606.5 112382250

18 dimethylaniline monooxygenase [N-oxide-forming] 3 60033.2 50541961

19 NADH-cytochrome b5 reductase 3 isoform 2 31628.5 193794826

20 tubulin alpha-1A chain 50135.3 17986283

21 dimethylaniline monooxygenase [N-oxide-forming] 5 isoform 1 60220.2 221316672

22 methyltransferase-like protein 7A precursor 28318.9 89145417

23 ras GTPase-activating-like protein IQGAP2 180577.0 116089337

24 amine oxidase [flavin-containing] B 58762.6 38202207

25 trifunctional enzyme subunit alpha, mitochondrial precursor 82999.1 20127408

26 carbamoyl-phosphate synthase [ammonia], mitochondrial isoform c 116037.4 170295797

27 cytochrome P450 2C19 precursor 55944.8 4503219

28 peroxisomal multifunctional enzyme type 2 isoform 1 83024.9 313482810

29 perilipin-2 48075.1 34577059

30 cytochrome P450 2C9 precursor 55627.6 13699818

31 acetyl-CoA carboxylase 2 precursor 276537.6 134142062

32 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581

33 fatty aldehyde dehydrogenase isoform 1 57669.1 73466520

34 ATP-binding cassette sub-family D member 3 isoform a 75475.5 4506341

35 peroxisomal bifunctional enzyme isoform 2 69153.5 261878539

36 cytochrome P450 2A7 isoform 2 precursor 50666.4 15147328

37 serum albumin preproprotein 69366.4 4502027

38 very long-chain acyl-CoA synthetase isoform 1 70311.8 227499619

39 UDP-glucuronosyltransferase 2B4 precursor 60512.1 149944509

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40 protein ERGIC-53 precursor 57548.6 5031873

41 estradiol 17-beta-dehydrogenase 11 32935.6 142976729

42 ATP synthase subunit beta, mitochondrial precursor 56559.5 32189394

43 endoplasmin precursor 92468.2 4507677

44 monoglyceride lipase isoform 1 34292.3 6005786

45 perilipin-3 isoform 1 47074.7 255958282

46 apolipoprotein A-I preproprotein 30777.6 4557321

47 coatomer subunit alpha isoform 2 138344.7 148536853

48 calnexin precursor 67567.8 10716563

49 arylacetamide deacetylase 45733.4 68299767

50 UDP-glucuronosyltransferase 2B15 precursor 61036.0 116517299

51 protein disulfide-isomerase precursor 57115.9 20070125

52 plectin isoform 1e 513705.9 41322908

53 synaptic vesicle membrane protein VAT-1 homolog 41920.0 18379349

54 cullin-associated NEDD8-dissociated protein 1 136374.7 21361794

55 liver carboxylesterase 1 isoform c precursor 62392.5 68508957

56 spectrin alpha chain, brain isoform 1 285091.3 194595509

57 cytoplasmic dynein 1 heavy chain 1 532406.6 33350932

58 apolipoprotein E precursor 36153.8 4557325

59 tubulin beta chain 49670.5 29788785

60 sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating 41900.0 8393516

61 microsomal glutathione S-transferase 1 17598.6 9945306

62 NAD(P) transhydrogenase, mitochondrial precursor 113894.8 122939155

63 serum paraoxonase/arylesterase 1 precursor 39731.0 19923106

64 cytochrome P450 2C8 isoform b 47785.8 311893309

65 cytochrome P450 1A2 58407.1 73915100

66 78 kDa glucose-regulated protein precursor 72332.5 16507237

67 annexin A6 isoform 1 75872.8 71773329

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68 adipocyte plasma membrane-associated protein 46480.1 24308201

69 PREDICTED: liver carboxylesterase 1-like 27540.5 341916335

70 redox-regulatory protein PAMM isoform 1 precursor 25764.0 344925828

71 retinol dehydrogenase 16 35673.2 150247226

72 dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48

kDa subunit precursor 50701.3 20070197

73 vimentin 53651.3 62414289

74 ATP-dependent RNA helicase DDX3X isoform 2 73156.0 301171467

75 voltage-dependent anion-selective channel protein 1 30772.3 4507879

76 cytochrome P450 3A7 57525.2 262290932

77 very long-chain specific acyl-CoA dehydrogenase, mitochondrial

isoform 2 precursor 68058.1 76496475

78 C-1-tetrahydrofolate synthase, cytoplasmic 101530.5 222136639

79 MOSC domain-containing protein 2, mitochondrial precursor 38023.2 31542713

80 probable saccharopine dehydrogenase 47151.1 55770836

81 cytochrome P450 2A6 precursor 56517.1 189339233

82 carnitine O-palmitoyltransferase 1, liver isoform isoform 1 88367.1 73623030

83 3-hydroxyacyl-CoA dehydratase 3 43159.3 117168248

84 delta(24)-sterol reductase precursor 60100.9 13375618

85 ATP-binding cassette sub-family A member 6 184284.2 27436953

86 histone H4 11367.3 4504301

87 bile salt sulfotransferase 33779.6 29540545

88 amine oxidase [flavin-containing] A 59681.3 4557735

89 alcohol dehydrogenase 1B 39835.3 34577061

90 perilipin-1 55990.0 223718203

91 alpha-actinin-4 104853.3 12025678

92 succinate dehydrogenase [ubiquinone] flavoprotein subunit,

mitochondrial 72691.1 156416003

93 elongation factor 1-alpha 2 50469.8 4503475

94 aldehyde dehydrogenase family 8 member A1 isoform 3 48035.6 301500698

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95 protein diaphanous homolog 1 isoform 1 141346.4 119395758

96 dolichyl-diphosphooligosaccharide--protein glycosyltransferase

subunit 1 precursor 68569.0 4506675

97 estradiol 17-beta-dehydrogenase 12 34323.9 7705855

98 ribosome-binding protein 1 108654.8 110611220

99 17-beta-hydroxysteroid dehydrogenase type 6 35965.5 19743808

100 dolichyl-diphosphooligosaccharide--protein glycosyltransferase

subunit 2 isoform 1 precursor 69283.5 35493916

101 trifunctional enzyme subunit beta, mitochondrial precursor 51294.1 4504327

102 programmed cell death 6-interacting protein isoform 1 96022.6 22027538

103 myosin-Ic isoform c 117906.3 124494240

104 elongation factor 2 95337.6 4503483

105 bifunctional ATP-dependent dihydroxyacetone kinase/FAD-AMP

lyase (cyclizing) 58946.8 20149621

106 myosin-9 226529.8 12667788

107 prenylcysteine oxidase 1 precursor 56639.8 166795301

108 SEC14-like protein 2 isoform 1 46145.0 7110715

109 D-3-phosphoglycerate dehydrogenase 56650.2 23308577

110 long-chain-fatty-acid--CoA ligase 5 isoform a 82262.5 42794756

111 serum paraoxonase/lactonase 3 39607.2 29788996

112 glutamate dehydrogenase 2, mitochondrial precursor 61433.6 31377775

113 sarcoplasmic/endoplasmic reticulum calcium ATPase 2 isoform b 114756.1 24638454

114 PREDICTED: POTE ankyrin domain family member I isoform 2 121281.5 88953571

115 retinol dehydrogenase 10 38087.3 25282469

116 glycerol-3-phosphate acyltransferase 1, mitochondrial precursor 93794.2 190358539

117 transferrin receptor protein 2 isoform 2 69617.1 332309171

118 cytochrome P450 2C18 isoform 1 precursor 55710.4 13699816

119 alpha-mannosidase 2 131139.6 51477714

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120 histone H2B type 1-C/E/F/G/I 13906.0 4504271

121 17-beta-hydroxysteroid dehydrogenase 13 isoform A 33655.2 210032110

122 cytochrome P450 4F11 precursor 60145.5 193083178

123 protein transport protein Sec24A isoform 1 119748.6 116174780

124 dehydrogenase/reductase SDR family member 7 precursor 38298.5 7706318

125 vitronectin precursor 54305.3 88853069

126 UDP-glucuronosyltransferase 1-6 isoform 1 precursor 60750.4 45827765

127 cytochrome P450 2A13 56687.2 13699809

128 Golgi apparatus protein 1 isoform 2 precursor 135994.8 224586815

129 calcium-binding mitochondrial carrier protein Aralar1 74761.4 21361103

130 perilipin-4 134430.4 122937195

131 methyltransferase-like protein 7B precursor 27774.6 164663805

132 mitochondrial carrier homolog 2 33330.7 7657347

133 histone H2A type 1-D 14107.4 10800130

134 actin, cytoplasmic 1 41736.5 4501885

135 7-dehydrocholesterol reductase 54489.1 119943112

136 peroxisomal multifunctional enzyme type 2 isoform 2 79685.9 4504505

137 myosin-Ib isoform 2 124949.7 44889481

138 reticulon-4 isoform A 129930.3 24431935

139 tripeptidyl-peptidase 1 preproprotein 61247.6 5729770

140 prohibitin-2 isoform 1 33296.1 221307584

141 cytochrome b-c1 complex subunit 1, mitochondrial precursor 52645.5 46593007

142 coatomer subunit beta 107141.5 7705369

143 prolow-density lipoprotein receptor-related protein 1 precursor 504608.8 126012562

144 ras-related protein Rab-14 23896.8 19923483

145 60S ribosomal protein L18 21634.3 4506607

146 kynurenine 3-monooxygenase 55809.7 187960045

147 cytochrome P450 4A22 59245.5 62952506

148 ras-related protein Rab-7a 23489.6 34147513

149 importin-5 125544.3 24797086

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150 UDP-glucuronosyltransferase 1-4 precursor 60024.7 6005930

151 40S ribosomal protein SA 32853.8 59859885

152 ras-related protein Rap-1b isoform 2 16033.3 354459350

153 ATP synthase subunit O, mitochondrial precursor 23277.2 4502303

154 guanine nucleotide-binding protein subunit beta-2-like 1 35076.5 5174447

155 transmembrane 9 superfamily member 2 precursor 75775.3 4758874

156 long-chain-fatty-acid--CoA ligase 3 80419.6 42794752

157 cathepsin D preproprotein 44552.0 4503143

158 apolipoprotein(a) precursor 226543.6 116292750

159 sarcoplasmic/endoplasmic reticulum calcium ATPase 3 isoform a 109255.5 28373103

160 hemoglobin subunit delta 16055.4 4504351

161 60 kDa heat shock protein, mitochondrial 61054.2 31542947

162 nodal modulator 1 precursor 134323.0 51944953

163 hemoglobin subunit beta 15998.3 4504349

164 cytochrome P450 4A11 59347.5 158937242

165 cytochrome P450 2A7 isoform 1 precursor 56424.8 15147330

166 ras-related protein Rab-2B isoform 2 19005.1 254281345

167 peroxisomal membrane protein 2 22252.4 8923892

168 melanoma inhibitory activity protein 3 precursor 213699.7 122891870

169 corticosteroid 11-beta-dehydrogenase isozyme 1 32400.8 5031765

170 coatomer subunit gamma 97717.7 11559929

171 cytochrome b5 isoform 1 15330.0 41281768

172 receptor expression-enhancing protein 6 20733.0 19923919

173 erlin-2 isoform 1 37839.3 6005721

174 epoxide hydrolase 2 62615.4 27597073

175 protein disulfide-isomerase A4 precursor 72931.9 4758304

176 transmembrane protein 205 21197.7 63055043

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177 leukotriene-B(4) omega-hydroxylase 1 precursor 59853.2 13435391

178 signal transducer and activator of transcription 3 isoform 1 88067.3 21618340

179 extended synaptotagmin-1 isoform 2 122855.6 14149680

180 alpha-actinin-1 isoform b 103056.9 4501891

181 lysophospholipid acyltransferase 5 56034.7 42542394

182 Golgi integral membrane protein 4 81879.8 7657138

183 cytochrome P450 3A5 isoform 1 57108.3 4503231

184 abhydrolase domain-containing protein 14B 22345.5 14249382

185 phosphate carrier protein, mitochondrial isoform b precursor 39958.5 47132595

186 exportin-1 123385.0 4507943

187 apoptosis-inducing factor 1, mitochondrial isoform 2 precursor 66294.3 22202629

188 tubulin alpha-1B chain 50151.3 57013276

189 general vesicular transport factor p115 107906.7 4505541

190 endoplasmic reticulum-Golgi intermediate compartment protein 1 32592.1 72534712

191 dynamin-1-like protein isoform 1 81876.7 171460914

192 uncharacterized protein C9orf174 197342.3 302565871

193 vesicular integral-membrane protein VIP36 precursor 40228.4 5803023

194 protein transport protein Sec23A 86160.3 38202214

195 beta-galactoside alpha-2,6-sialyltransferase 1 isoform a 46604.3 27765091

196 beta-2-glycoprotein 1 precursor 38298.0 153266841

197 UDP-glucuronosyltransferase 2B10 isoform 2 precursor 50688.0 221219059

198 L-xylulose reductase isoform 2 25742.7 304571975

199 clusterin preproprotein 52494.3 355594753

200 reticulon-4 isoform C 22395.3 5902016

201 acid ceramidase isoform b 46503.3 189011546

202 cytochrome b5 type B 16694.4 83921614

203 calpain-1 catalytic subunit 81889.4 12408656

204 ADP/ATP translocase 2 32852.0 156071459

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205 E3 UFM1-protein ligase 1 89594.6 24308039

206 voltage-dependent anion-selective channel protein 3 isoform 2 30789.6 208879465

207 vacuolar protein sorting-associated protein 13C isoform 1B 403082.1 66348091

208 protein disulfide-isomerase A6 precursor 48121.0 5031973

209 Golgi-specific brefeldin A-resistance guanine nucleotide exchange

factor 1 isoform 1 206443.7 4758416

210 60S ribosomal protein L7 29225.6 15431301

211 cytochrome P450 4F8 precursor 59994.3 6005737

212 immunoglobulin-like and fibronectin type III domain-containing

protein 1 383803.9 257196151

213 cytochrome b-c1 complex subunit 2, mitochondrial precursor 48442.7 50592988

214 poly [ADP-ribose] polymerase 14 202798.2 154813199

215 RIB43A-like with coiled-coils protein 1 isoform 1 44014.7 72534784

216 electron transfer flavoprotein-ubiquinone oxidoreductase,

mitochondrial precursor 68495.0 119703746

217 erythrocyte band 7 integral membrane protein isoform a 31730.6 38016911

218 membrane-associated progesterone receptor component 1 21671.0 5729875

219 tubulin beta-2C chain 49830.7 5174735

220 apolipoprotein C-III precursor 10852.2 4557323

221 estradiol 17-beta-dehydrogenase 2 42784.9 4504503

222 hydroxymethylglutaryl-CoA synthase, mitochondrial isoform 1

precursor 56635.1 5031751

223 protein PRRC1 46700.9 18677735

224 POTE ankyrin domain family member F 121443.8 153791352

225 AP-2 complex subunit beta isoform b 104551.9 4557469

226 plasminogen isoform 1 precursor 90568.6 4505881

227 40S ribosomal protein S5 22876.3 13904870

228 heat shock protein HSP 90-alpha isoform 1 98160.4 153792590

Page 23: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

229 heterogeneous nuclear ribonucleoprotein U isoform a 90584.0 74136883

230 sorbitol dehydrogenase 38324.3 156627571

231 surfeit locus protein 4 30393.8 19557691

232 UDP-N-acetylglucosamine transporter 35984.2 6912668

233 coatomer subunit gamma-2 97621.8 109134349

234 sec1 family domain-containing protein 1 isoform a 72379.4 33469966

235 leucine-rich repeat-containing protein 59 34930.2 40254924

236 heterogeneous nuclear ribonucleoprotein H2 49263.3 9624998

237 retinol dehydrogenase 11 isoform 1 precursor 35386.0 166795268

238 ATP-binding cassette sub-family D member 1 82936.5 7262393

239 dual specificity mitogen-activated protein kinase kinase 2 44423.9 13489054

240 60S ribosomal protein L4 47697.0 16579885

241 sulfotransferase 1A2 34310.4 4507303

242 40S ribosomal protein S3a 29944.7 4506723

243 ras-related protein Rab-8A 23668.1 16933567

244 phosphoglucomutase-1 isoform 1 61448.7 21361621

245 aldehyde oxidase 147916.9 71773480

246 heat shock protein HSP 90-beta 83263.6 20149594

247 transmembrane protein 33 27978.0 224589127

248 myosin-Ia 118399.8 4885503

249 60S ribosomal protein L19 23465.8 4506609

250 microsomal glutathione S-transferase 3 16516.2 4758714

251 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10,

mitochondrial precursor 40750.3 4758768

252 40S ribosomal protein S3 26688.1 15718687

253 catechol O-methyltransferase isoform MB-COMT 30036.9 4502969

254 alpha-soluble NSF attachment protein 33232.5 47933379

255 junction plakoglobin 81744.3 12056468

256 probable ATP-dependent RNA helicase DDX5 69147.6 4758138

257 40S ribosomal protein S8 24205.0 4506743

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258 mevalonate kinase 42450.6 4557769

259 voltage-dependent anion-selective channel protein 2 isoform 1 33371.4 296317337

260 TRAM adaptor with GOLD domain isoform 2 precursor 21232.8 256985102

261 non-specific lipid-transfer protein isoform 1 proprotein 58993.3 19923233

262 E3 ubiquitin-protein ligase MARCH5 31231.5 8923415

263 heterogeneous nuclear ribonucleoprotein F 45671.6 4826760

264 long-chain-fatty-acid--CoA ligase 6 isoform d 77670.3 327412323

265 multidrug resistance-associated protein 6 isoform 1 164872.8 190343023

266 acetolactate synthase-like protein 67867.3 21361361

267 catenin alpha-1 100070.6 55770844

268 cytochrome P450 3A4 isoform 2 57255.8 322302351

269 vesicle-associated membrane protein-associated protein B/C isoform

1 27228.2 4759302

270 UDP-glucuronosyltransferase 1-9 precursor 59940.7 11276085

271 formimidoyltransferase-cyclodeaminase 58926.2 11140815

272 carbamoyl-phosphate synthase [ammonia], mitochondrial isoform a

precursor 165648.8 169790915

273 26S proteasome non-ATPase regulatory subunit 5 56195.4 4826952

274 vesicle transport protein GOT1B 15425.6 7705636

275 UDP-glucuronosyltransferase 2B11 precursor 61037.9 4507823

276 aldehyde dehydrogenase family 8 member A1 isoform 1 53401.1 12007648

277 60S ribosomal protein L23 14865.4 4506605

278 eukaryotic initiation factor 4A-II 46402.0 83700235

279 3-ketoacyl-CoA thiolase, mitochondrial 41923.9 167614485

280 C4b-binding protein alpha chain precursor 67032.9 4502503

281 hemoglobin subunit alpha 15257.4 4504345

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282 N-terminal kinase-like protein isoform A 89630.9 115430241

283 protein MON2 homolog 190356.9 114326552

284 mannosyl-oligosaccharide glucosidase isoform 1 91917.0 149999606

285 lanosterol synthase isoform 1 83308.3 47933395

286 ras-related protein Rab-18 22976.9 10880989

287 epidermal growth factor receptor kinase substrate 8-like protein 2 80620.2 21264616

288 neutral cholesterol ester hydrolase 1 isoform c 31167.6 226423950

289 14-3-3 protein epsilon 29173.7 5803225

290 acetyl-CoA acetyltransferase, mitochondrial precursor 45199.3 4557237

291 3-ketoacyl-CoA thiolase, peroxisomal isoform a 44291.8 4501853

292 transmembrane 9 superfamily member 1 isoform b precursor 55250.5 62460635

293 asialoglycoprotein receptor 2 isoform a 35191.1 4502253

294 GTP-binding protein SAR1a 22366.6 217416369

295 ras-related protein Rab-10 22540.8 256222019

296 cutaneous T-cell lymphoma-associated antigen 9 87952.4 224586773

297 inositol 1,4,5-trisphosphate receptor type 2 308061.1 95147335

298 malectin precursor 32233.6 7661948

299 elongation factor 1-alpha 1 50140.5 4503471

300 ATP synthase subunit gamma, mitochondrial isoform H (heart)

precursor 32880.7 4885079

301 40S ribosomal protein S15a 14839.4 71772415

302 galectin-4 35940.9 5453712

303 transitional endoplasmic reticulum ATPase 89321.3 6005942

304 thromboxane-A synthase isoform 1 60649.2 195972898

305 apolipoprotein O-like precursor 29158.7 116812610

306 tubulin alpha chain-like 3 isoform 1 49908.5 13376181

307 S-adenosylmethionine synthase isoform type-1 43647.7 4557737

308 annexin A4 36084.8 4502105

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309 acyl-CoA dehydrogenase family member 11 87263.9 38505218

310 cytoskeleton-associated protein 4 66022.0 19920317

311 leukotriene-B(4) omega-hydroxylase 2 isoform a 59846.4 119220562

312 HLA class I histocompatibility antigen, A-1 alpha chain precursor 40840.5 24797067

313 leukotriene-B(4) omega-hydroxylase 2 isoform b 59738.4 312836833

314 succinate dehydrogenase [ubiquinone] iron-sulfur subunit,

mitochondrial precursor 31629.6 115387094

315 histone H2B type 1-O 13906.0 16306566

316 dedicator of cytokinesis protein 7 239419.7 54112429

317 oligosaccharyltransferase complex subunit OSTC 16829.2 24308271

318 docking protein 1 isoform 1 52391.5 4503357

319 lanosterol 14-alpha demethylase isoform 1 precursor 57278.0 4503243

320 protein LYRIC 63836.5 223555917

321 translational activator GCN1 292707.0 54607053

322 histone H1.0 20862.8 4885371

323 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform

1 precursor 70729.6 66346721

324 ras-related protein Rap-1A 20987.1 4506413

325 fatty acid-binding protein, liver 14208.3 4557577

326 dynamin-like 120 kDa protein, mitochondrial isoform 1 111629.9 224831243

327 asialoglycoprotein receptor 1 isoform b 29143.8 308387363

328 guanine nucleotide-binding protein G(I)/G(S)/G(T) subunit beta-2 37330.8 20357529

329 cytochrome P450 3A43 isoform 2 57669.5 16933533

330 sterol 26-hydroxylase, mitochondrial precursor 60234.4 4503211

331 sodium- and chloride-dependent glycine transporter 1 isoform 3 70582.4 67782317

332 peroxisomal trans-2-enoyl-CoA reductase 32544.1 19923817

333 MAP kinase-activating death domain protein isoform g 175915.5 18860873

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334 prohibitin 29803.9 4505773

335 carnitine O-palmitoyltransferase 2, mitochondrial precursor 73776.6 4503023

336 15-hydroxyprostaglandin dehydrogenase [NAD+] isoform 1 28977.1 31542939

337 D-beta-hydroxybutyrate dehydrogenase, mitochondrial precursor 38156.9 44680133

338 thioredoxin-related transmembrane protein 1 precursor 31791.0 151101292

339 erlin-1 39170.9 154800487

340 histone H3.1 15404.0 4504281

341 serine/threonine-protein kinase OSR1 58021.8 4826878

342 NADH-cytochrome b5 reductase 1 34094.6 49574502

343 40S ribosomal protein S2 31324.2 15055539

344 ATP-binding cassette sub-family A member 1 254298.9 21536376

345 proteasome activator complex subunit 2 27401.4 30410792

346 RPS10-NUDT3 protein 33140.6 321117084

347 rho-related GTP-binding protein RhoB precursor 22123.3 4757764

348 bax inhibitor 1 isoform 1 26537.6 148746209

349 atlastin-3 60541.6 45827806

350 importin subunit beta-1 97169.7 19923142

351 multivesicular body subunit 12B isoform 1 35619.4 58761488

352 type I iodothyronine deiodinase isoform a 28774.2 4557522

353 26S protease regulatory subunit 10B 45796.7 195539395

354 desmoplakin isoform I 331771.4 58530840

355 serine/threonine-protein phosphatase PGAM5, mitochondrial

isoform 1 32004.2 281604136

356 transmembrane emp24 domain-containing protein 9 precursor 27277.2 39725636

357 2,4-dienoyl-CoA reductase, mitochondrial precursor 36067.5 4503301

358 dolichyl-diphosphooligosaccharide--protein glycosyltransferase

subunit DAD1 12496.6 4503253

359 peroxisomal bifunctional enzyme isoform 1 79494.4 68989263

360 phosphoinositide 3-kinase adapter protein 1 90397.3 45505139

361 stomatin-like protein 2 38533.8 7305503

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362 trifunctional purine biosynthetic protein adenosine-3 isoform 1 107766.5 209869993

363 UDP-glucuronosyltransferase 2B28 isoform 2 precursor 38743.0 333033811

364 UPF0554 protein C2orf43 37318.3 11345458

365 puromycin-sensitive aminopeptidase 103275.6 158937236

366 calreticulin precursor 48141.1 4757900

367 palmitoyl-protein thioesterase 1 isoform 1 precursor 34193.3 4506031

368 ras-related protein Rap-1b isoform 4 15355.6 354459356

369 brefeldin A-inhibited guanine nucleotide-exchange protein 2 202036.4 150417986

370 fibrinogen gamma chain isoform gamma-A precursor 49496.2 70906437

371 3-keto-steroid reductase 38205.9 7705421

372 vacuolar protein sorting-associated protein 13A isoform C 355859.0 66346672

373 cytochrome c oxidase subunit 7A2, mitochondrial precursor 12843.9 262118227

374 protein FAM5B precursor 89004.1 23943866

375 polypeptide N-acetylgalactosaminyltransferase 2 precursor 64732.4 4758412

376 alpha-tocopherol transfer protein 31749.4 4507723

377 translocon-associated protein subunit alpha precursor 32235.1 169404009

378 UBX domain-containing protein 4 56777.2 24307965

379 tudor domain-containing protein 6 isoform 1 236514.7 58197558

380 probable N-acetyltransferase 8 25618.9 117190517

381 arginase-1 isoform 2 34734.7 10947139

382 translocation protein SEC63 homolog 87996.4 6005872

383 pre-mRNA cleavage complex 2 protein Pcf11 173048.7 33620745

384 Golgin subfamily B member 1 376015.7 148596984

385 ras-related protein Rab-11A isoform 1 24393.3 4758984

386 protein NipSnap homolog 1 isoform 1 33309.7 193211616

387 talin-1 269764.4 223029410

388 signal transducer and activator of transcription 6 isoform 2 81747.4 296010868

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389 sorting nexin-14 isoform a 110180.9 24797145

390 ragulator complex protein LAMTOR1 17744.7 8923579

391 alpha-1,3-mannosyl-glycoprotein 2-beta-N-

acetylglucosaminyltransferase 50878.0 167857778

392 40S ribosomal protein S7 22126.7 4506741

393 cathepsin B preproprotein 37821.4 4503139

394 lysosome-associated membrane glycoprotein 2 isoform A precursor 44960.5 4504957

395 60S acidic ribosomal protein P0 34273.3 16933546

396 Golgi pH regulator B 52916.7 7706704

397 coiled-coil domain-containing protein 47 precursor 55873.5 171906582

398 60S ribosomal protein L18a 20762.2 11415026

399 apolipoprotein L3 isoform 1 44277.9 22035646

400 DNA ligase 4 103970.1 23199993

401 hemoglobin subunit epsilon 16202.7 4885393

402 5'-AMP-activated protein kinase subunit beta-2 30302.0 4885561

403 proteasome activator complex subunit 1 isoform 1 28722.9 5453990

404 7-alpha-hydroxycholest-4-en-3-one 12-alpha-hydroxylase 58068.1 166362724

405 signal peptidase complex catalytic subunit SEC11A 20625.2 7657609

406 conserved oligomeric Golgi complex subunit 7 86343.4 23957690

407 neuropathy target esterase isoform b 146214.6 116256487

408 probable E3 ubiquitin-protein ligase HERC6 isoform 1 115125.5 61563742

409 torsin-1A-interacting protein 1 66248.0 39753957

410 mitogen-activated protein kinase kinase kinase 15 147436.0 282847398

411 lipopolysaccharide-responsive and beige-like anchor protein isoform

2 319105.2 148233596

412 tyrosine-protein kinase BAZ1B 170901.2 14670392

413 signal recognition particle receptor subunit alpha isoform 1 69810.8 23308697

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414 RANBP2-like and GRIP domain-containing protein 8 198990.7 256600210

415 cytochrome c oxidase subunit II 25564.8 251831110

416 transient receptor potential cation channel subfamily M member 2 171224.5 4507689

417 serum paraoxonase/arylesterase 2 isoform 1 39380.6 66529294

418 serine beta-lactamase-like protein LACTB, mitochondrial isoform a 60693.3 26051231

419 neuron navigator 3 253019.3 120953251

420 mesoderm induction early response protein 2 59943.9 45267839

421 tricarboxylate transport protein, mitochondrial precursor 34012.4 21389315

422 alcohol dehydrogenase 1C 39867.4 4501933

423 HLA class I histocompatibility antigen, Cw-1 alpha chain precursor 40648.3 52630342

424 protein transport protein Sec24B isoform a 137416.6 112382212

425 E3 ubiquitin-protein ligase TRIM23 isoform alpha 64066.3 4502197

426 guanine nucleotide-binding protein G(i) subunit alpha-2 isoform 1 40450.7 4504041

427 ras GTPase-activating-like protein IQGAP1 189250.1 4506787

428 chromosome-associated kinesin KIF4A 139880.1 116686122

429 gamma-parvin 37485.0 11545879

430 centrosomal protein of 128 kDa 128014.2 64085252

431 gamma-aminobutyric acid receptor subunit beta-2 isoform 1

precursor 59150.1 12548785

432 ribonuclease P protein subunit p40 41833.7 55743132

433 enoyl-CoA hydratase domain-containing protein 1 isoform 1 32997.0 157694516

434 dynein heavy chain 14, axonemal isoform 1 518563.2 223555935

435 zinc finger and BTB domain-containing protein 1 isoform 2 73682.1 182509178

436 inositol 1,4,5-trisphosphate receptor type 1 isoform 1 308536.7 269954690

437 myosin-14 isoform 3 232008.3 224831241

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438 transmembrane protein 109 precursor 26209.8 13129092

439 cystatin-9-like precursor 17275.7 18104940

440 testis-expressed sequence 2 protein 125997.8 38679909

441 cytochrome P450 4F22 61957.9 158138530

442 NEDD8 ultimate buster 1 isoform 2 71921.4 343403796

443 A disintegrin and metalloproteinase with thrombospondin motifs 20

preproprotein 214718.3 124430557

444 annexin A3 36375.0 4826643

445 heat shock 70 kDa protein 1A/1B 70051.8 194248072

446 outer dense fiber protein 2 isoform 1 103066.6 310750400

447 titin isoform N2-B 2992860.0 291045223

448 cadherin-4 isoform 2 96396.6 356640221

449 killer cell lectin-like receptor subfamily F member 1 26562.4 7705574

450 uncharacterized protein KIAA2026 228084.7 148612838

451 guanylate cyclase soluble subunit alpha-3 isoform A 77452.1 67763816

452 ATPase family AAA domain-containing protein 5 207568.0 26080431

453 coiled-coil domain-containing protein 164 87133.6 217416374

454 DNA-binding protein RFXANK isoform a 28102.1 4506499

455 olfactory receptor 5B3 35257.5 53793671

456 oxidoreductase HTATIP2 isoform b 27048.8 148728164

457 myotilin isoform a 55394.8 5803106

458 charged multivesicular body protein 5 isoform 1 24570.6 189409150

459 muskelin isoform 1 82404.6 223890246

460 serine/threonine-protein kinase pim-2 34190.1 42821112

461 nucleoprotein TPR 267289.5 114155142

462 POU domain, class 5, transcription factor 2 36050.9 23463326

463 plexin-B1 precursor 232295.2 40254442

464 MIT domain-containing protein 1 29314.2 20270349

465 exportin-5 136310.3 22748937

466 aminopeptidase Q 113281.8 194239713

467 prelamin-A/C isoform 1 precursor 74139.0 27436946

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468 WD repeat-containing protein 88 52620.7 102470001

469 microtubule-actin cross-linking factor 1 isoform a 620419.4 33188445

470 DNA repair protein RAD52 homolog 46168.4 109637798

471 canalicular multispecific organic anion transporter 2 isoform 2 64631.5 221316556

472 receptor-type tyrosine-protein phosphatase delta isoform 1 precursor 214757.5 4506309

473 gamma-tubulin complex component 2 102533.3 5729840

474 filamin-A-interacting protein 1 138108.1 31542634

475 calmodulin-regulated spectrin-associated protein 2 166730.9 44955929

476 cytochrome P450 2B6 precursor 56278.0 7949031

477 ankyrin-3 isoform 1 480407.6 32967601

478 transmembrane emp24 domain-containing protein 4 precursor 25942.6 33457308

479 ankyrin repeat domain-containing protein 34C 58256.0 226437606

480 beta-defensin 116 precursor 11544.3 83582800

481 leiomodin-2 61675.0 150378503

482 cyclic nucleotide-gated cation channel alpha-3 isoform 1 78837.6 4502917

483 tripartite motif-containing protein 65 57353.1 38679905

484 oxidation resistance protein 1 isoform 3 97969.4 309384265

485 helicase SRCAP 343551.4 146219843

486 centromere protein J 152999.0 130980075

487 pleckstrin homology domain-containing family G member 5 isoform

c 117450.4 111154078

488 DENN domain-containing protein 4B 163845.5 148922920

489 syntaxin-binding protein 4 61661.4 63999048

490 FH1/FH2 domain-containing protein 3 160802.3 58331242

491 polycomb protein SCMH1 isoform f 52908.0 288557339

492 potassium/sodium hyperpolarization-activated cyclic nucleotide-

gated channel 2 96950.0 156071470

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493 potassium voltage-gated channel subfamily G member 2 51239.3 6912444

494 histone deacetylase 9 isoform 4 117206.8 30795202

495 DNA repair protein REV1 isoform 1 138247.1 7706681

496 disabled homolog 2 isoform 1 82447.3 148491082

497 aldose reductase 35853.2 4502049

498 insulin receptor-related protein precursor 143719.0 31657140

499 integrin alpha-4 precursor 114898.8 67191027

500 6-phosphofructokinase type C isoform 2 85315.5 334191701

501 serotransferrin precursor 77049.5 4557871

502 sperm protein associated with the nucleus on the X chromosome N4 11168.3 57528117

503 NEDD8-MDP1 protein 22027.0 315259111

504 fructose-bisphosphate aldolase B 39472.8 40354205

505 proline-rich protein 12 211041.2 153792074

506 methylmalonate-semialdehyde dehydrogenase [acylating],

mitochondrial precursor 57839.4 11095441

507 arf-GAP with SH3 domain, ANK repeat and PH domain-containing

protein 2 isoform a 111649.9 4502249

508 ESF1 homolog 98795.5 18093112

509 NKG2-A/NKG2-B type II integral membrane protein isoform

NKG2-B 24222.6 7262384

510 F-box/WD repeat-containing protein 7 isoform 1 79662.5 16117781

511 leucine-rich repeats and immunoglobulin-like domains protein 3

isoform 1 precursor 117437.1 209862903

512 biliverdin reductase A precursor 33428.3 33589854

513 tubulin beta-3 chain isoform 1 50432.4 50592996

514 cytochrome P450 2F1 precursor 55501.0 19743565

515 transformation/transcription domain-associated protein isoform 1 437598.6 347360922

516 SH3 domain and tetratricopeptide repeats-containing protein 1 146944.2 145386551

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517 phosphatidylinositol 3,4,5-trisphosphate-dependent Rac exchanger 1

protein 186202.7 34452732

518 lysophosphatidylcholine acyltransferase 2 60207.5 47086907

519 coiled-coil domain-containing protein 110 isoform a 96724.8 22749515

520 A-kinase anchor protein 9 isoform 3 452107.5 22538393

521 extended synaptotagmin-2 98901.0 45387945

522 SH3 domain-containing kinase-binding protein 1 isoform a 73125.9 13994242

523 coiled-coil domain-containing protein 34 isoform 2 26255.8 18087817

524 dynein heavy chain 7, axonemal 461158.7 151301127

525 myosin-10 228996.6 41406064

526 arginyl-tRNA synthetase, cytoplasmic 75378.5 15149476

527 proteoglycan 4 isoform A precursor 151075.6 67190163

528 nucleotide-binding oligomerization domain-containing protein 1 107690.5 5174617

529 lanC-like protein 2 50854.1 8923911

530 probable ubiquitin carboxyl-terminal hydrolase FAF-X isoform 3 292276.7 145309309

531 whirlin isoform 1 96557.1 290746376

532 centrosome-associated protein 350 350927.9 171184451

533 complexin-4 18336.4 31795561

534 HAUS augmin-like complex subunit 5 71682.1 149944680

535 outer dense fiber protein 2-like isoform a 71961.8 296179395

536 myosin light chain kinase, smooth muscle isoform 2 203053.5 116008188

537 sterile alpha motif domain-containing protein 9 184278.9 38201706

538 uncharacterized protein C3orf19 53957.2 108773808

539 protein furry homolog 338871.6 117606355

540 alpha-mannosidase 2C1 115834.5 46852164

541 methionine synthase 140526.2 169790923

542 ankyrin-3 isoform 2 111097.9 32967599

543 tubulin polyglutamylase TTLL5 143576.5 50658079

Page 35: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

544 autism susceptibility gene 2 protein isoform 1 138981.3 17864090

545 zinc finger protein 34 64038.1 157954634

546 ras-related protein Rab-8B 23584.0 7706563

547 transducin beta-like protein 2 precursor 49797.6 7549793

548 zinc finger CCHC domain-containing protein 4 59009.6 148886718

549 spectrin beta chain, erythrocyte isoform a 267823.1 67782321

550 janus kinase and microtubule-interacting protein 2 94933.3 45237195

551 thyroid adenoma-associated protein 219604.8 38348727

552 midasin 632819.0 24415404

553 pentatricopeptide repeat-containing protein 3, mitochondrial

precursor 78549.2 38683855

554 toll-like receptor 1 precursor 90290.8 41350337

555 myosin light chain 5 19533.9 4505305

Table S4. Identification of proteins associated with LDs of human liver sample 3.

Reference MW Accession

Scan(s) Sp RSp

1 17-beta-hydroxysteroid dehydrogenase 13 isoform B 29604.7 210032112

2 fatty acid synthase 273423.2 41872631

3 ras GTPase-activating-like protein IQGAP2 180577.0 116089337

4 spectrin beta chain, brain 1 isoform 1 274606.5 112382250

5 very long-chain specific acyl-CoA dehydrogenase, mitochondrial

isoform 2 precursor 68058.1 76496475

6 carbamoyl-phosphate synthase [ammonia], mitochondrial isoform c 116037.4 170295797

7 serum albumin preproprotein 69366.4 4502027

8 epoxide hydrolase 1 precursor 52948.5 209862837

9 alcohol dehydrogenase 4 40221.5 71565152

10 estradiol 17-beta-dehydrogenase 11 32935.6 142976729

11 perilipin-2 48075.1 34577059

12 clathrin heavy chain 1 191612.6 4758012

13 spectrin alpha chain, brain isoform 1 285091.3 194595509

14 methyltransferase-like protein 7A precursor 28318.9 89145417

Page 36: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

15 myosin-9 226529.8 12667788

16 ATP synthase subunit beta, mitochondrial precursor 56559.5 32189394

17 keratin, type I cytoskeletal 18 48057.5 4557888

18 keratin, type II cytoskeletal 1 66038.3 119395750

19 long-chain-fatty-acid--CoA ligase 1 77942.9 40807491

20 probable saccharopine dehydrogenase 47151.1 55770836

21 keratin, type I cytoskeletal 10 58800.6 195972866

22 dehydrogenase/reductase SDR family member 1 33908.8 209862901

23 protein diaphanous homolog 1 isoform 1 141346.4 119395758

24 peroxisomal multifunctional enzyme type 2 isoform 1 83024.9 313482810

25 tubulin beta-2A chain 49906.7 4507729

26 fructose-bisphosphate aldolase B 39472.8 40354205

27 ATP synthase subunit alpha, mitochondrial precursor 59750.3 4757810

28 trifunctional enzyme subunit alpha, mitochondrial precursor 82999.1 20127408

29 formimidoyltransferase-cyclodeaminase 58926.2 11140815

30 bile acyl-CoA synthetase precursor 75384.7 13325057

31 complement C3 precursor 187146.3 115298678

32 glycogen debranching enzyme isoform 1 174762.1 116734847

33 alpha-actinin-4 104853.3 12025678

34 lanosterol synthase isoform 2 82198.1 224177556

35 long-chain-fatty-acid--CoA ligase 5 isoform a 82262.5 42794756

36 tubulin alpha-1A chain 50135.3 17986283

37 17-beta-hydroxysteroid dehydrogenase 13 isoform A 33655.2 210032110

38 cullin-associated NEDD8-dissociated protein 1 136374.7 21361794

39 calnexin precursor 67567.8 10716563

40 annexin A6 isoform 1 75872.8 71773329

41 prelamin-A/C isoform 1 precursor 74139.0 27436946

42 UTP--glucose-1-phosphate uridylyltransferase isoform a 56939.9 48255966

43 fatty aldehyde dehydrogenase isoform 1 57669.1 73466520

44 cytochrome P450 2C9 precursor 55627.6 13699818

45 dimethylaniline monooxygenase [N-oxide-forming] 3 60033.2 50541961

46 bile salt sulfotransferase 33779.6 29540545

47 monoglyceride lipase isoform 1 34292.3 6005786

48 perilipin-1 55990.0 223718203

49 elongation factor 2 95337.6 4503483

50 talin-1 269764.4 223029410

51 78 kDa glucose-regulated protein precursor 72332.5 16507237

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52 C-1-tetrahydrofolate synthase, cytoplasmic 101530.5 222136639

53 endoplasmin precursor 92468.2 4507677

54 bifunctional ATP-dependent dihydroxyacetone kinase/FAD-AMP lyase

(cyclizing) 58946.8 20149621

55 keratin, type II cytoskeletal 8 53703.9 4504919

56 betaine--homocysteine S-methyltransferase 1 44998.2 157266337

57 peroxisomal bifunctional enzyme isoform 2 69153.5 261878539

58 NADH-cytochrome b5 reductase 3 isoform 2 31628.5 193794826

59 neutral alpha-glucosidase AB isoform 3 precursor 109437.2 88900491

60 protein disulfide-isomerase precursor 57115.9 20070125

61 programmed cell death 6-interacting protein isoform 1 96022.6 22027538

62 perilipin-4 134430.4 122937195

63 PREDICTED: liver carboxylesterase 1-like 27540.5 341916335

64 plectin isoform 1e 513705.9 41322908

65 protein ERGIC-53 precursor 57548.6 5031873

66 NADPH--cytochrome P450 reductase 77047.7 127139033

67 perilipin-3 isoform 1 47074.7 255958282

68 alcohol dehydrogenase 1B 39835.3 34577061

69 aldehyde oxidase 147916.9 71773480

70 apolipoprotein B-100 precursor 515528.6 105990532

71 protein-glutamine gamma-glutamyltransferase 2 isoform a 77328.4 39777597

72 carnitine O-palmitoyltransferase 2, mitochondrial precursor 73776.6 4503023

73 sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating 41900.0 8393516

74 ribosome-binding protein 1 108654.8 110611220

75 long-chain-fatty-acid--CoA ligase 3 80419.6 42794752

76 protein transport protein Sec23A 86160.3 38202214

77 retinol dehydrogenase 10 38087.3 25282469

78 general vesicular transport factor p115 107906.7 4505541

79 carbamoyl-phosphate synthase [ammonia], mitochondrial isoform a

precursor 165648.8 169790915

80 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit

2 isoform 1 precursor 69283.5 35493916

81 keratin, type I cytoskeletal 9 62063.9 55956899

82 liver carboxylesterase 1 isoform c precursor 62392.5 68508957

83 D-3-phosphoglycerate dehydrogenase 56650.2 23308577

84 hypoxia up-regulated protein 1 precursor 111334.8 5453832

Page 38: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

85 cytosolic 10-formyltetrahydrofolate dehydrogenase 98828.6 21614513

86 prohibitin-2 isoform 1 33296.1 221307584

87 microsomal triglyceride transfer protein large subunit precursor 99350.6 153285408

88 alcohol dehydrogenase 1C 39867.4 4501933

89 coatomer subunit beta 107141.5 7705369

90 arylacetamide deacetylase 45733.4 68299767

91 succinate dehydrogenase [ubiquinone] flavoprotein subunit,

mitochondrial 72691.1 156416003

92 amine oxidase [flavin-containing] A 59681.3 4557735

93 enoyl-CoA hydratase, mitochondrial precursor 31387.2 194097323

94 actin, cytoplasmic 1 41736.5 4501885

95 alcohol dehydrogenase [NADP+] 36572.8 24497577

96 sorbitol dehydrogenase 38324.3 156627571

97 phenylalanine-4-hydroxylase 51861.8 4557819

98 aldehyde dehydrogenase family 8 member A1 isoform 3 48035.6 301500698

99 dimethylaniline monooxygenase [N-oxide-forming] 5 isoform 1 60220.2 221316672

100 tripeptidyl-peptidase 1 preproprotein 61247.6 5729770

101 dehydrogenase/reductase SDR family member on chromosome X

precursor 36442.9 193804850

102 hydroxymethylglutaryl-CoA synthase, mitochondrial isoform 1

precursor 56635.1 5031751

103 AP-2 complex subunit beta isoform b 104551.9 4557469

104 hemoglobin subunit alpha 15257.4 4504345

105 cytochrome P450 2C8 isoform b 47785.8 311893309

106 elongation factor 1-alpha 2 50469.8 4503475

107 acetyl-CoA acetyltransferase, mitochondrial precursor 45199.3 4557237

108 voltage-dependent anion-selective channel protein 1 30772.3 4507879

109 arginase-1 isoform 2 34734.7 10947139

110 T-complex protein 1 subunit zeta isoform a 58023.9 4502643

111 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581

112 aldo-keto reductase family 1 member C1 36788.1 5453543

113 cytochrome P450 4A11 59347.5 158937242

114 argininosuccinate lyase isoform 1 51657.7 68303542

115 nicotinamide phosphoribosyltransferase precursor 55520.8 5031977

116 L-serine dehydratase/L-threonine deaminase 34625.1 33469958

117 heterogeneous nuclear ribonucleoprotein K isoform a 51027.9 14165437

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118 UDP-glucuronosyltransferase 2B7 precursor 60720.3 190194389

119 alpha-mannosidase 2 131139.6 51477714

120 junction plakoglobin 81744.3 12056468

121 glutamate dehydrogenase 2, mitochondrial precursor 61433.6 31377775

122 5'-AMP-activated protein kinase subunit gamma-1 isoform 1 37579.1 4506061

123 protein transport protein Sec24A isoform 1 119748.6 116174780

124 non-specific lipid-transfer protein isoform 1 proprotein 58993.3 19923233

125 catenin beta-1 85496.2 4503131

126 UDP-glucuronosyltransferase 1-1 precursor 59591.1 8850236

127 glutamate dehydrogenase 1, mitochondrial precursor 61397.5 4885281

128 carbonyl reductase [NADPH] 1 30374.7 4502599

129 fructose-1,6-bisphosphatase 1 36842.3 189083692

130 1-acylglycerol-3-phosphate O-acyltransferase ABHD5 39095.4 31542303

131 very long-chain acyl-CoA synthetase isoform 1 70311.8 227499619

132 short-chain dehydrogenase/reductase 3 33548.2 31543615

133 plastin-2 70288.0 167614506

134 malate dehydrogenase, cytoplasmic isoform 2 36425.9 5174539

135 alpha-1-antitrypsin precursor 46736.2 50363217

136 reticulon-4 isoform A 129930.3 24431935

137 argininosuccinate synthase 46530.1 16950633

138 heat shock protein HSP 90-alpha isoform 1 98160.4 153792590

139 guanine nucleotide-binding protein subunit beta-2-like 1 35076.5 5174447

140 guanine nucleotide-binding protein G(I)/G(S)/G(T) subunit beta-2 37330.8 20357529

141 exportin-2 110415.8 29029559

142 filamin-B isoform 2 278160.3 105990514

143 alpha-soluble NSF attachment protein 33232.5 47933379

144 amine oxidase [flavin-containing] B 58762.6 38202207

145 villin-1 92694.4 194394237

146 corticosteroid 11-beta-dehydrogenase isozyme 1 32400.8 5031765

147 ubiquitin-like modifier-activating enzyme 1 117848.1 23510340

148 cytochrome b-c1 complex subunit 2, mitochondrial precursor 48442.7 50592988

149 transitional endoplasmic reticulum ATPase 89321.3 6005942

150 ancient ubiquitous protein 1 45786.6 31712030

151 vimentin 53651.3 62414289

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152 phosphoglucomutase-1 isoform 1 61448.7 21361621

153 radixin 68563.4 4506467

154 long-chain-fatty-acid--CoA ligase 4 isoform 2 79187.6 12669909

155 regulator of G-protein signaling 14 61446.6 21361304

156 nesprin-1 isoform 1 1011101.0 330688408

157 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform 1

precursor 70729.6 66346721

158 RIB43A-like with coiled-coils protein 1 isoform 1 44014.7 72534784

159 keratin, type II cytoskeletal 2 epidermal 65432.4 47132620

160 calpain-1 catalytic subunit 81889.4 12408656

161 tubulin beta-2C chain 49830.7 5174735

162 rab GDP dissociation inhibitor alpha 50582.4 4503971

163 protein disulfide-isomerase A3 precursor 56782.0 21361657

164 glyceraldehyde-3-phosphate dehydrogenase 36053.0 7669492

165 short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

precursor 47485.1 4501859

166 fibrinogen gamma chain isoform gamma-A precursor 49496.2 70906437

167 UBX domain-containing protein 4 56777.2 24307965

168 retinal dehydrogenase 1 54861.5 21361176

169 membrane primary amine oxidase 84621.4 4502119

170 inter-alpha-trypsin inhibitor heavy chain H1 isoform a 101388.4 156119625

171 catenin alpha-1 100070.6 55770844

172 exportin-1 123385.0 4507943

173 2,4-dienoyl-CoA reductase, mitochondrial precursor 36067.5 4503301

174 apoptosis-inducing factor 2 40526.4 14318424

175 annexin A2 isoform 2 38603.8 209862831

176 GDH/6PGL endoplasmic bifunctional protein precursor 88892.2 52145310

177 hemoglobin subunit delta 16055.4 4504351

178 cystathionine beta-synthase 60586.2 4557415

179 rRNA 2'-O-methyltransferase fibrillarin 33784.0 12056465

180 erythrocyte band 7 integral membrane protein isoform a 31730.6 38016911

181 myosin-10 228996.6 41406064

182 serum paraoxonase/arylesterase 1 precursor 39731.0 19923106

183 keratin, type I cytoskeletal 16 51267.5 24430192

184 microsomal glutathione S-transferase 1 17598.6 9945306

185

dihydrolipoyllysine-residue succinyltransferase component of 2-

oxoglutarate dehydrogenase complex, mitochondrial isoform 1

precursor

48755.0 19923748

186 coatomer subunit gamma-2 97621.8 109134349

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187 large proline-rich protein BAG6 isoform a 119408.2 149158692

188 thymidine phosphorylase precursor 49955.2 166158922

189 cytochrome P450 4F8 precursor 59994.3 6005737

190 gamma-glutamyltransferase 5 isoform b 62260.8 153266885

191 SEC14-like protein 3 46047.9 27923592

192 3-ketoacyl-CoA thiolase, peroxisomal isoform a 44291.8 4501853

193 prohibitin 29803.9 4505773

194 tubulin alpha-1B chain 50151.3 57013276

195 cytochrome P450 2D6 isoform 1 55730.1 40805836

196 UDP-glucose 6-dehydrogenase isoform 1 55023.8 4507813

197 leukotriene-B(4) omega-hydroxylase 1 precursor 59853.2 13435391

198 estradiol 17-beta-dehydrogenase 2 42784.9 4504503

199 SEC14-like protein 2 isoform 1 46145.0 7110715

200 estradiol 17-beta-dehydrogenase 12 34323.9 7705855

201 gap junction beta-1 protein 32024.3 4504005

202 ras-related protein Rab-14 23896.8 19923483

203 PRA1 family protein 3 21614.6 5453704

204 B-cell receptor-associated protein 31 isoform b 27991.4 213511012

205 RNA-binding protein EWS isoform 2 68477.8 4885225

206 bile acid-CoA:amino acid N-acyltransferase 46298.9 4502351

207 ras-related protein Rap-1b isoform 2 16033.3 354459350

208 aldehyde dehydrogenase, mitochondrial isoform 1 precursor 56380.9 25777732

209 serine/threonine-protein kinase PAK 2 58042.3 32483399

210 acetolactate synthase-like protein 67867.3 21361361

211 glucose-6-phosphate isomerase isoform 2 63146.7 18201905

212 proteasome activator complex subunit 1 isoform 1 28722.9 5453990

213 L-lactate dehydrogenase A chain isoform 1 36688.5 5031857

214 ubiquitin-60S ribosomal protein L40 precursor 14728.2 77539055

215 histone H2A type 1-D 14107.4 10800130

216 myeloperoxidase precursor 83868.1 4557759

217 annexin A5 35936.5 4502107

218 short-chain specific acyl-CoA dehydrogenase, mitochondrial precursor 44297.0 4557233

219 epoxide hydrolase 2 62615.4 27597073

220 OCIA domain-containing protein 1 isoform 1 27626.0 8923427

221 UDP-glucuronosyltransferase 1-7 precursor 59818.5 41282213

222 ATP-dependent RNA helicase DDX3X isoform 2 73156.0 301171467

223 COP9 signalosome complex subunit 7a 30276.4 7705330

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224 protein odr-4 homolog isoform 1 51102.8 57527756

225 lanosterol synthase isoform 1 83308.3 47933395

226 cytochrome P450 2E1 precursor 56848.6 10834998

227 UDP-glucuronosyltransferase 2B15 precursor 61036.0 116517299

228 cytoplasmic aconitate hydratase 98398.2 8659555

229 3-mercaptopyruvate sulfurtransferase isoform 2 33178.2 61835204

230 alpha-enolase isoform 1 47168.7 4503571

231 ER lumen protein retaining receptor 2 isoform 1 24421.7 5803050

232 carbonic anhydrase 2 29245.8 4557395

233 cathepsin B preproprotein 37821.4 4503139

234 alcohol dehydrogenase 6 isoform 2 39072.4 4501939

235 aldo-keto reductase family 1 member C4 37094.6 325652083

236 chromosome-associated kinesin KIF4A 139880.1 116686122

237 60S ribosomal protein L27 15797.6 4506623

238 HLA class I histocompatibility antigen, Cw-1 alpha chain precursor 40648.3 52630342

239 importin subunit beta-1 97169.7 19923142

240 glutathione S-transferase kappa 1 isoform a 25496.7 7705704

241 scavenger receptor cysteine-rich type 1 protein M130 isoform a

precursor 125450.2 344179110

242 vacuolar protein sorting-associated protein 13C isoform 1B 403082.1 66348091

243 lamin-B1 isoform 1 66407.9 5031877

244 S-adenosylmethionine synthase isoform type-1 43647.7 4557737

245 macrosialin isoform A precursor 37407.9 91199548

246 band 3 anion transport protein 101791.6 4507021

247 fibrinogen beta chain isoform 1 preproprotein 55927.8 70906435

248 1,4-alpha-glucan-branching enzyme 80459.3 189458812

249 ATP synthase subunit d, mitochondrial isoform a 18491.1 5453559

250 obg-like ATPase 1 isoform 1 44743.2 58761500

251 selenium-binding protein 1 52390.7 16306550

252 vigilin isoform b 137969.8 345199283

253 ras-related protein Rab-10 22540.8 256222019

254 4-aminobutyrate aminotransferase, mitochondrial precursor 56438.6 38679946

255 receptor expression-enhancing protein 6 20733.0 19923919

256 peroxisomal bifunctional enzyme isoform 1 79494.4 68989263

257 alanine aminotransferase 1 54636.6 4885351

258 eukaryotic translation initiation factor 3 subunit A 166568.5 4503509

259 peroxisomal trans-2-enoyl-CoA reductase 32544.1 19923817

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260 heat shock protein HSP 90-beta 83263.6 20149594

261 Golgi apparatus protein 1 isoform 2 precursor 135994.8 224586815

262 high mobility group protein B1 24893.6 4504425

263 lipopolysaccharide-binding protein precursor 53383.3 31652249

264 staphylococcal nuclease domain-containing protein 1 101996.3 77404397

265 annexin A1 38714.0 4502101

266 beta-2-glycoprotein 1 precursor 38298.0 153266841

267 heterogeneous nuclear ribonucleoprotein A3 39594.6 34740329

268 cytochrome P450 2A7 isoform 2 precursor 50666.4 15147328

269 coatomer subunit alpha isoform 2 138344.7 148536853

270 apolipoprotein E precursor 36153.8 4557325

271 myosin-Ic isoform c 117906.3 124494240

272 3 beta-hydroxysteroid dehydrogenase type 7 isoform b 21322.2 218563684

273 target of Myb protein 1 isoform 1 53818.1 4885637

274 eukaryotic translation initiation factor 3 subunit L isoform 1 66726.6 7705433

275 UDP-glucuronosyltransferase 1-4 precursor 60024.7 6005930

276 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit

1 precursor 68569.0 4506675

277 microsomal glutathione S-transferase 3 16516.2 4758714

278 beta,beta-carotene 9',10'-oxygenase isoform a 65673.7 82617624

279 surfeit locus protein 4 30393.8 19557691

280 synaptic vesicle membrane protein VAT-1 homolog 41920.0 18379349

281 guanine nucleotide-binding protein G(i) subunit alpha-2 isoform 1 40450.7 4504041

282 aminopeptidase N precursor 109538.8 157266300

283 aspartyl/asparaginyl beta-hydroxylase isoform f 83267.4 258613943

284 prenylcysteine oxidase 1 precursor 56639.8 166795301

285 ras-related protein Rab-7a 23489.6 34147513

286 dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa

subunit precursor 50701.3 20070197

287 aflatoxin B1 aldehyde reductase member 3 37206.2 41152114

288 sodium-coupled neutral amino acid transporter 3 55772.8 5870893

289 signal transducer and activator of transcription 3 isoform 1 88067.3 21618340

290 plasminogen isoform 1 precursor 90568.6 4505881

291 catenin delta-1 isoform 2A 98048.4 332688222

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292 mitochondrial inner membrane protein isoform 1 83677.3 154354964

293 peroxiredoxin-4 precursor 30539.7 5453549

294 ATP-binding cassette sub-family D member 3 isoform a 75475.5 4506341

295 heterogeneous nuclear ribonucleoprotein U isoform a 90584.0 74136883

296 spectrin beta chain, brain 2 271291.8 5902122

297 serine--pyruvate aminotransferase 43009.7 4557289

298 proteasome inhibitor PI31 subunit 29816.5 145611426

299 rab GDP dissociation inhibitor beta isoform 1 50662.9 6598323

300 catalase 59755.8 4557014

301 oligosaccharyltransferase complex subunit OSTC 16829.2 24308271

302 patatin-like phospholipase domain-containing protein 3 52865.1 17196626

303 keratin, type II cytoskeletal 6A 60044.6 5031839

304 trifunctional enzyme subunit beta, mitochondrial precursor 51294.1 4504327

305 HCLS1-binding protein 3 42780.0 68800430

306 cytochrome P450 3A43 isoform 2 57669.5 16933533

307 39S ribosomal protein L28, mitochondrial precursor 30156.6 26051229

308 60S acidic ribosomal protein P0 34273.3 16933546

309 fibrinogen alpha chain isoform alpha-E preproprotein 94972.5 4503689

310 pyruvate carboxylase, mitochondrial precursor 129632.7 106049292

311 vacuolar protein sorting-associated protein VTA1 homolog 33879.0 21361741

312 7-alpha-hydroxycholest-4-en-3-one 12-alpha-hydroxylase 58068.1 166362724

313 heterogeneous nuclear ribonucleoprotein C-like 1 32142.2 61966711

314 cAMP-dependent protein kinase catalytic subunit alpha isoform 1 40589.3 4506055

315 C4b-binding protein alpha chain precursor 67032.9 4502503

316 mitochondrial glutamate carrier 1 34470.0 13375983

317 histamine N-methyltransferase isoform 1 33294.9 5901970

318 dynamin-2 isoform 5 97976.6 299758394

319 alpha-actinin-1 isoform b 103056.9 4501891

320 sulfotransferase 1A2 34310.4 4507303

321 vacuolar protein sorting-associated protein 35 91706.5 17999541

322 D-beta-hydroxybutyrate dehydrogenase, mitochondrial precursor 38156.9 44680133

323 protein FAM5B precursor 89004.1 23943866

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324 vitamin K-dependent gamma-carboxylase isoform 1 87560.4 21361163

325 guanine nucleotide-binding protein G(i) subunit alpha-1 40360.8 33946324

326 ankyrin repeat and FYVE domain-containing protein 1 isoform 1 128485.4 110815813

327 apolipoprotein L3 isoform 1 44277.9 22035646

328 uridine 5'-monophosphate synthase 52221.3 4507835

329 POTE ankyrin domain family member F 121443.8 153791352

330 UDP-glucose:glycoprotein glucosyltransferase 1 precursor 177187.7 9910280

331 inverted formin-2 isoform 1 135623.5 149999380

332 gamma-glutamyl hydrolase precursor 35964.1 4503987

333 UDP-glucuronosyltransferase 2A2 60771.7 333609245

334 UDP-glucuronosyltransferase 1-9 precursor 59940.7 11276085

335 AP-3 complex subunit beta-1 121319.4 32484979

336 peroxiredoxin-6 25034.8 4758638

337 F-actin-capping protein subunit beta isoform 2 31350.3 330864679

338 protein NDRG2 isoform a 40797.9 42544211

339 hydroxyacid oxidase 1 40924.1 11068137

340 complement factor B preproprotein 85532.4 67782358

341 UDP-glucuronosyltransferase 2B28 isoform 2 precursor 38743.0 333033811

342 myosin-Ib isoform 2 124949.7 44889481

343 probable ATP-dependent RNA helicase DDX17 isoform 1 80272.0 38201710

344 mitochondrial import receptor subunit TOM40 homolog 37892.8 5174723

345 cadherin-4 isoform 2 96396.6 356640221

346 6-phosphofructokinase, liver type 85017.9 48762920

347 heterogeneous nuclear ribonucleoproteins A2/B1 isoform A2 36005.7 4504447

348 alpha-2-macroglobulin precursor 163290.5 66932947

349 elongation factor Tu, mitochondrial precursor 49874.6 34147630

350 phosphatidylinositol-binding clathrin assembly protein isoform 1 70754.1 56788366

351 EH domain-containing protein 3 60886.7 7657056

352 alpha-1,3-mannosyl-glycoprotein 2-beta-N-

acetylglucosaminyltransferase 50878.0 167857778

353 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9,

mitochondrial precursor 42509.3 6681764

354 17-beta-hydroxysteroid dehydrogenase type 6 35965.5 19743808

355 cytochrome P450 2C19 precursor 55944.8 4503219

356 metal transporter CNNM2 isoform 3 60487.1 40068051

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357 splicing factor 3B subunit 1 isoform 1 145829.1 54112117

358 cytochrome P450 4F11 precursor 60145.5 193083178

359 ATP-binding cassette sub-family F member 1 isoform a 95925.1 69354671

360 E3 ubiquitin-protein ligase TTC3 229865.8 49640009

361 hemoglobin subunit epsilon 16202.7 4885393

362 RNA-binding protein 33 129985.5 151301053

363 apolipoprotein A-I preproprotein 30777.6 4557321

364 cytochrome b5 isoform 1 15330.0 41281768

365 rho GTPase-activating protein 35 170512.4 150417981

366 signal recognition particle 68 kDa protein 70729.2 24497620

367 prostaglandin reductase 1 isoform 2 32894.9 226056130

368 beta-glucuronidase precursor 74731.4 268834192

369 caprin-2 isoform 2 120973.4 23503235

370 PREDICTED: LOW QUALITY PROTEIN: arf-GAP with GTPase,

ANK repeat and PH domain-containing protein 10 75384.5 239744064

371 keratin, type II cytoskeletal 5 62378.0 119395754

372 phosphorylase b kinase regulatory subunit beta isoform a 124883.5 4505783

373 desmoplakin isoform I 331771.4 58530840

374 rho GTPase-activating protein 1 50435.4 4757766

375 acyl-coenzyme A synthetase ACSM5, mitochondrial precursor 64759.7 38505220

376 monoacylglycerol lipase ABHD12 isoform b 45558.0 24308097

377 ras-related protein Rab-2B isoform 2 19005.1 254281345

378 WD repeat-containing protein 7 isoform 1 163809.1 73747877

379 intraflagellar transport protein 172 homolog 197573.7 46358428

380 heat shock protein beta-1 22782.3 4504517

381 bactericidal permeability-increasing protein precursor 53899.4 157276599

382 prolargin precursor 43809.6 4506041

383 dual specificity mitogen-activated protein kinase kinase 1 43438.8 5579478

384 ADP/ATP translocase 1 33064.2 55749577

385 squalene monooxygenase 63922.8 62865635

386 60S ribosomal protein L30 12784.0 4506631

387 heat shock 70 kDa protein 1A/1B 70051.8 194248072

388 mitochondrial ribonuclease P protein 3 precursor 67315.0 62988276

389 melanoma inhibitory activity protein 3 precursor 213699.7 122891870

390 alpha-tocopherol transfer protein-like 38514.9 85861250

391 protein disulfide-isomerase A6 precursor 48121.0 5031973

392 sarcoplasmic/endoplasmic reticulum calcium ATPase 2 isoform b 114756.1 24638454

393 acid ceramidase isoform b 46503.3 189011546

394 glutamyl aminopeptidase 109243.5 132814467

Page 47: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

395 elongation factor 1-gamma 50118.5 4503481

396 dual specificity testis-specific protein kinase 2 63639.2 110349799

397 protein PRRC1 46700.9 18677735

398 serotransferrin precursor 77049.5 4557871

399 60 kDa heat shock protein, mitochondrial 61054.2 31542947

400 calcium-binding mitochondrial carrier protein Aralar1 74761.4 21361103

401 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit

STT3A 80529.1 22749415

402 nuclear receptor ROR-beta 52072.3 19743907

403 WASH complex subunit strumpellin 134285.3 120952851

404 kelch-like protein 34 70611.6 23397572

405 histone H4 11367.3 4504301

406 cytochrome P450 2A13 56687.2 13699809

407 baculoviral IAP repeat-containing protein 3 68371.2 33946285

408 vesicle-associated membrane protein-associated protein A isoform 1 32613.3 94721250

409 rhophilin-1 73589.5 119120915

410 vitronectin precursor 54305.3 88853069

411 bifunctional UDP-N-acetylglucosamine 2-epimerase/N-

acetylmannosamine kinase isoform 1 83065.5 190014632

412 glutathione S-transferase A3 25301.5 24430144

413 protein transport protein Sec24B isoform a 137416.6 112382212

414 L-lactate dehydrogenase A-like 6B 41942.7 15082234

415 cytochrome P450 4A22 59245.5 62952506

416 zinc finger and BTB domain-containing protein 34 55533.8 149944640

417 myosin-14 isoform 3 232008.3 224831241

418 acyl-CoA dehydrogenase family member 9, mitochondrial precursor 68760.0 21361497

419 translational activator GCN1 292707.0 54607053

420 carbonyl reductase [NADPH] 3 30850.1 4502601

421 DNA-directed RNA polymerase III subunit RPC2 isoform 2 121119.4 238908505

422 fibroblast growth factor 10 precursor 23435.8 4758360

423 uncharacterized protein C9orf174 197342.3 302565871

424 protocadherin Fat 2 precursor 479314.7 13787217

425 T-complex protein 1 subunit alpha isoform a 60343.2 57863257

426 PREDICTED: complement C4-A isoform 1 192843.7 341916194

427 myosin-VI 148712.8 92859701

428 nitric oxide synthase, brain isoform 1 160968.7 10835173

429 thiosulfate sulfurtransferase 33428.7 17402865

430 striatin-3 isoform 2 77744.2 142976675

431 transient receptor potential cation channel subfamily M member 1

isoform 1 186543.7 354721145

Page 48: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

432 tumor necrosis factor receptor superfamily member 19 isoform 2

precursor 45305.2 23238204

433 TFIIH basal transcription factor complex helicase XPD subunit isoform

1 86908.7 15834617

434 protein QN1 homolog 161941.6 148612801

435 leukocyte immunoglobulin-like receptor subfamily B member 5 isoform

3 precursor 52968.7 125987593

436 prolactin receptor isoform 6 precursor 57952.2 324073295

437 myotilin isoform a 55394.8 5803106

438 ribosomal protein S6 kinase-like 1 60035.9 332801047

439 E3 ubiquitin-protein ligase TRIM22 isoform 1 56947.0 117938316

440 Golgi integral membrane protein 4 81879.8 7657138

441 E3 ubiquitin-protein ligase HERC2 527227.1 126032348

442 laminin subunit alpha-2 isoform a precursor 343931.7 28559088

443 progesterone-induced-blocking factor 1 89804.0 55769583

444 fibrous sheath-interacting protein 2 789892.5 297206791

445 kinesin-like protein KIF14 186489.8 7661878

446 zinc finger protein 655 isoform a 57406.7 28416427

447 cytochrome P450 3A5 isoform 1 57108.3 4503231

448 protein-L-isoaspartate O-methyltransferase domain-containing protein 1 40675.0 190360566

449 plasminogen activator inhibitor 1 isoform 1 precursor 45059.7 10835159

450 parathyroid hormone 2 receptor precursor 62235.5 4826954

451 phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha

isoform 124283.3 54792082

452 myotubularin-related protein 2 isoform 1 73380.8 44680154

453 FYVE, RhoGEF and PH domain-containing protein 5 159889.7 194018497

454 PREDICTED: macrophage mannose receptor 1-like 166044.1 341915493

455 serine/threonine-protein kinase 38 54189.9 6005814

456 uncharacterized protein KIAA2026 228084.7 148612838

457 leiomodin-2 61675.0 150378503

458 vacuolar protein sorting-associated protein 37D 27729.6 117938318

459 cytochrome P450 2J2 57610.3 18491008

460 scavenger mRNA-decapping enzyme DcpS 38608.5 7661734

461 serpin B4 44853.7 28076869

462 adenylosuccinate synthetase isozyme 2 50097.0 34577063

463 attractin isoform 1 preproprotein 158535.6 21450861

464 cullin-5 90954.7 40254446

465 keratin, type II cytoskeletal 4 56143.7 331999954

466 ATPase family AAA domain-containing protein 2 158552.9 24497618

Page 49: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

467 calmodulin-regulated spectrin-associated protein 2 166730.9 44955929

468 adenylate cyclase type 5 isoform 1 138906.6 34486092

469 spondin-1 precursor 90973.2 110347423

470 cytochrome P450 1A2 58407.1 73915100

471 stress-associated endoplasmic reticulum protein 2 7430.9 58219012

472 39S ribosomal protein L55, mitochondrial isoform a 15128.3 31563362

473 triple functional domain protein 346897.7 45439359

474 structural maintenance of chromosomes protein 2 135655.0 110347420

475 telomerase protein component 1 290486.4 21536371

476 protein PAT1 homolog 2 61463.6 223278366

477 small G protein signaling modulator 2 isoform 1 118306.7 148612795

478 zinc finger protein 556 51580.9 13376460

479 proteasome activator complex subunit 4 211331.6 163644283

480 integrator complex subunit 2 134345.5 24308211

481 peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase isoform

1 74389.7 21314690

482 zinc finger protein 488 36961.8 23308499

483 FERM, RhoGEF and pleckstrin domain-containing protein 2 119887.7 7662310

484 creatine kinase B-type 42644.0 21536286

485 zinc finger protein 622 54271.8 15529978

486 80 kDa MCM3-associated protein 218402.0 19923191

487 PWWP domain-containing protein MUM1L1 79039.5 289577100

488 kinesin-like protein KIF24 151902.2 154426306

489 activating signal cointegrator 1 complex subunit 2 isoform 1 86359.9 20270253

490 receptor tyrosine-protein kinase erbB-2 isoform a precursor 137909.8 54792096

491 ubiquitin carboxyl-terminal hydrolase isozyme L1 24824.2 21361091

492 DNA-binding protein SATB1 isoform 1 85956.6 4506791

493 lactoylglutathione lyase 20777.6 118402586

494 protein FAM135A isoform c 169838.1 242117946

495 cystin-1 16393.0 81158080

496 centromere protein F 357524.8 55770834

497 U5 small nuclear ribonucleoprotein 200 kDa helicase 244504.7 40217847

498 putative uncharacterized protein C16orf96 125040.6 222446620

499 spectrin beta chain, erythrocyte isoform a 267823.1 67782321

500 glycerol kinase 2 60593.5 41393575

501 5-azacytidine-induced protein 1 isoform a 121846.8 111955084

Page 50: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

Table S5. Combined list of of proteins associated with LDs found in 3 human livers.

Reference MW Accession

Peptid

e (Hits)

Scan(s),

File Peptide

Sp RSp Ions

1 apolipoprotein B-100 precursor 515528.6

10599053

2

24 (21

3 0 0 0)

4118, 2-2 R.IGQDGISTSATTNLK.C 704.1 1 19/28

4608, 2-2 R.SEILAHWSPAK.L 2114.9 1 18/20

4980, 2-2 K.SGSSTASWIQNVDTK.Y 1145.6 1 16/28

4980, 2-2 K.HIYAISSAALSASYK.A 877.1 2 17/28

5142, 1-2 K.SGSSTASWIQNVDTK.Y 895.9 2 16/28

5470, 1-2 R.DLKVEDIPLAR.I 1555.5 1 16/20

5543, 2-2 R.EYSGTIASEANTYLNSK.S 1038.1 1 19/32

5684, 2-2 K.IAELSATAQEIIK.S 1570.3 1 18/24

5816, 2-2 K.ALYWVNGQVPDGVSK.V 553.0 1 14/28

6062, 2-2 K.ALVEQGFTVPEIK.T 714.3 1 17/24

6251, 2-2 R.LPYTIITTPPLK.D 1162.2 1 17/22

6280, 2-2 R.TEHGSEMLFFGNAIEGK.S 684.2 1 17/32

6436, 2-2 R.VIGNMGQTMEQLTPELK.S 751.0 1 17/32

6495, 2-2 K.IDDIWNLEVK.E 880.0 1 15/18

6612, 1-2 R.TGISPLALIK.G 806.6 1 16/18

6780, 2-2 R.LAAYLMLMR.S 1231.3 1 14/16

6891, 2-2 R.VLLDQLGTTISFER.I 688.5 2 16/26

7120, 2-2 K.NLTDFAEQYSIQDWAK.R 907.7 1 17/30

7125, 2-2 K.NLTDFAEQYSIQDWAK.R 2353.8 1 23/30

7190, 1-2 K.DKDQEVLLQTFLDDASPGDKR.L 1272.9 1 31/80

7690, 2-2 R.NIQEYLSILTDPDGK.G 925.0 1 17/28

8259, 1 R.ILGEELGFASLHDLQLLGK.L 1690.2 1 23/36

8775, 1 R.VPSYTLILPSLELPVLHVPR.N 1101.3 1 30/76

8944, 2-2 K.IAIANIIDEIIEK.L 857.5 1 16/24

2 clathrin heavy chain 1 191612.6 4758012

34 (34

0 0 0 0)

4258, 1-2 K.VIQCFAETGQVQK.I 2175.5 1 19/24

4452, 2-2 R.HSSLAGCQIINYR.T 1335.9 1 18/24

4454, 2-2 R.HSSLAGCQIINYR.T 658.0 1 15/24

4570, 1-2 R.HSSLAGCQIINYR.T 1442.7 1 18/24

4767, 2-2 K.VANVELYYR.A 1047.2 1 14/16

4928, 1-2 K.VSQPIEGHAASFAQFK.M 1385.0 1 20/30

4964, 2-2 R.KFNALFAQGNYSEAAK.V 1728.8 1 23/30

5110, 1-2 R.KFNALFAQGNYSEAAK.V 1616.6 1 22/30

5177, 1-2 K.IVLDNSVFSEHR.N 674.1 1 14/22

5388, 1-2 R.ALEHFTDLYDIKR.A 1107.6 1 17/24

5469, 1 K.LLYNNVSNFGR.L 1020.4 1 15/20

5616, 1-2 K.LHIIEVGTPPTGNQPFPK.K 563.6 1 20/34

5628, 1-2 K.LHIIEVGTPPTGNQPFPK.K 601.7 1 19/34

5834, 2-2 R.NNLAGAEELFAR.K 1396.4 1 18/22

5979, 1-2 R.NNLAGAEELFAR.K 1272.9 1 18/22

6070, 1 R.NNLAGAEELFAR.K 1097.8 1 15/22

6087, 1-2 R.GQFSTDELVAEVEKR.N 1086.9 1 20/28

Page 51: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

6290, 1-2 R.TSIDAYDNFDNISLAQR.L 2157.2 1 23/32

6834, 2-2 K.WLLLTGISAQQNR.V 1618.0 1 18/24

6857, 2-2 R.GQCDLELINVCNENSLFK.S 1246.5 1 23/34

7042, 1-2 K.WLLLTGISAQQNR.V 1735.6 1 19/24

7079, 1-2 R.GQCDLELINVCNENSLFK.S 859.3 1 20/34

7235, 2-2 K.SVNESLNNLFITEEDYQALR.T 653.9 1 19/38

7481, 1-2 K.SVNESLNNLFITEEDYQALR.T 1308.9 1 23/38

7586, 1 K.SVDPTLALSVYLR.A 2152.4 1 20/24

7613, 1 K.SVNESLNNLFITEEDYQALR.T 1118.0 1 22/38

7731, 2-2 R.NLQNLLILTAIK.A 1702.5 1 18/22

7924, 1-2 K.KVGYTPDWIFLLR.N 1103.1 1 15/24

7996, 1-2 R.NLQNLLILTAIK.A 1651.9 1 18/22

8087, 1 R.NLQNLLILTAIK.A 837.7 1 17/22

8115, 1 R.RPLIDQVVQTALSETQDPEEVSVTVK.A

1191.0 1 34/100

8140, 1 R.LPVVIGGLLDVDCSEDVIK.N 1572.3 1 21/36

8698, 1-2 K.VGYTPDWIFLLR.N 512.9 1 15/22

8786, 1 K.VGYTPDWIFLLR.N 665.3 1 17/22

3 long-chain-fatty-acid--CoA ligase 1 77942.9 40807491

38 (38

0 0 0 0)

3767, 2-2 R.IFGQANTTLKR.W 986.0 1 16/20

3831, 1-2 R.IFGQANTTLKR.W 842.0 1 16/20

3959, 2-2 R.VVECVMLCHGAK.I 804.0 1 16/22

4028, 1-2 R.VVECVMLCHGAK.I 640.2 1 14/22

4095, 2-2 R.GIQVSNNGPCLGSR.K 1495.1 1 19/26

4260, 2-2 R.IFGQANTTLK.R 932.4 1 16/18

4413, 1-2 K.DSGLKPFEQVK.G 963.9 1 16/20

4877, 1-2 R.NIVSDCSAFVK.A 566.2 1 14/20

4893, 1-2 R.NIVSDCSAFVK.A 829.6 1 17/20

4995, 2-2 K.TAEALDKDGWLHTGDIGK.W 1918.1 1 21/34

5038, 2-2 K.RGFEGSFEELCR.N 937.7 1 15/22

5133, 1-2 K.TAEALDKDGWLHTGDIGK.W 2160.3 1 34/68

5262, 2-2 K.GPNVFQGYLKDPAK.T 909.4 1 17/26

5354, 1-2 K.GPNVFQGYLKDPAK.T 1330.3 1 21/26

5504, 2-2 R.KPDQPYEWLSYK.Q 1405.4 1 18/22

5523, 2-2 R.GFEGSFEELCR.N 1310.5 1 16/20

5613, 2-2 K.AELSLVFVDKPEK.A 1168.3 1 18/24

5657, 1-2 R.KPKPPAPEDLAVICFTSGTTGNPK.G

1354.8 1 32/92

5850, 1 K.AELSLVFVDKPEK.A 1026.5 1 17/24

5970, 1 K.IIVVM*DAYGSELVER.G 897.7 1 19/28

6063, 1 K.IGFFQGDIR.L 1126.7 1 14/16

6218, 2-2 K.QVAELSECIGSALIQK.G 877.1 1 27/60

6342, 2-2 R.SQIDDLYSTIKV.- 1368.1 1 17/22

6780, 1 K.IIVVMDAYGSELVER.G 548.5 1 17/28

7499, 2-2 K.GITLHPELFSIDNGLLTPTMK.A 640.5 1 17/40

7755, 1-2 K.GITLHPELFSIDNGLLTPTMK.A 999.1 1 24/40

7898, 2-2 R.LM*VTGAAPVSATVLTFLR.A 587.0 1 20/34

7905, 2-2 R.LM*VTGAAPVSATVLTFLR.A 1243.5 1 29/68

8169, 1-2 R.LM*VTGAAPVSATVLTFLR.A 749.0 1 21/34

8172, 1-2 R.LM*VTGAAPVSATVLTFLR.A 912.4 1 26/68

Page 52: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

8204, 2-2 R.LMVTGAAPVSATVLTFLR.A 707.9 1 23/34

8206, 2-2 R.LMVTGAAPVSATVLTFLR.A 1077.7 1 27/68

8211, 2-2 R.LMVTGAAPVSATVLTFLR.A 634.9 1 22/34

8291, 1 R.LM*VTGAAPVSATVLTFLR.A 951.2 1 24/34

8468, 1-2 R.LMVTGAAPVSATVLTFLR.A 998.4 1 26/34

8477, 1-2 R.LMVTGAAPVSATVLTFLR.A 1044.2 1 30/68

8576, 1 R.LMVTGAAPVSATVLTFLR.A 591.3 1 20/34

8583, 1 R.LMVTGAAPVSATVLTFLR.A 884.1 1 26/68

4 fatty acid synthase 273423.2 41872631

27 (27

0 0 0 0)

4343, 2-2 R.VTVAGGVHISGLHTESAPR.R 882.9 1 20/36

4632, 1 R.VTVAGGVHISGLHTESAPR.R 1086.3 1 28/72

4686, 1-2 K.EDGLAQQQTQLNLR.S 923.6 1 15/26

5024, 2-2 R.AALQEELQLCK.G 1447.8 1 15/20

5033, 2-2 R.LQVVDQPLPVR.G 930.0 1 14/20

5550, 1-2 R.FPQLDSTSFANSR.D 1923.2 1 20/24

5728, 1 K.TGGAYGEDLGADYNLSQVCDGK.V 1233.3 1 22/42

6488, 1 K.GVDLVLNSLAEEK.L 1449.0 1 18/24

6717, 2-2 K.AINCATSGVVGLVNCLR.R 1701.6 1 23/32

6728, 1 K.VVVQVLAEEPEAVLK.G 748.8 1 16/28

6748, 1-2 K.SFYGSTLFLCR.R 1330.8 1 15/20

6780, 1-2 R.LNSVQSSERPLFLVHPIEGSTTVFHSLASR.L

968.6 1 35/116

6818, 1 R.LNSVQSSERPLFLVHPIEGSTTVFHSLASR.L

1284.5 1 38/116

6854, 1 K.SFYGSTLFLCR.R 1482.9 1 17/20

7299, 1 R.IPGLLSPHPLLQLSYTATDR.H 2459.3 1 39/76

7689, 1 R.LHLSGIDANPNALFPPVEFPAPR.G 708.8 1 28/88

7712, 1 R.EGGFLLLHTLLR.G 713.7 1 13/22

7799, 2-2 R.DNLEFFLAGIGR.L 2101.9 1 19/22

8049, 1-2 R.DNLEFFLAGIGR.L 2659.6 1 19/22

8114, 2-2 K.LPEDPLLSGLLDSPALK.A 1568.6 1 23/32

8313, 1 R.EACPELDYFVVFSSVSCGR.G 731.2 1 17/36

8333, 1 R.EACPELDYFVVFSSVSCGR.G 967.2 1 19/36

8398, 1-2 K.LPEDPLLSGLLDSPALK.A 1263.9 1 22/32

9442, 2-2 R.DLVEAVAHILGIR.D 1063.8 1 16/24

9533, 1 R.TLLEGSGLESIISIIHSSLAEPR.V 581.1 1 17/44

9686, 1 R.DLVEAVAHILGIR.D 1387.0 1 19/24

9744, 1-2 R.DLVEAVAHILGIR.D 1454.1 1 18/24

5 ras GTPase-activating-like protein IQGAP2 180577.0

11608933

7

29 (29

0 0 0 0)

3755, 1-2 R.KSFLHEQEENVVK.I 1429.3 1 18/24

4283, 1-2 K.SFLHEQEENVVK.I 932.2 1 15/22

4511, 1-2 R.FQATSSGPILR.E 669.0 1 14/20

5034, 2-2 K.AWVNQLETQTGEASK.L 669.4 1 17/28

5193, 1-2 K.AWVNQLETQTGEASK.L 1600.2 1 20/28

5356, 1 K.AWVNQLETQTGEASK.L 838.5 1 17/28

5372, 1 K.SKVDQVQDIVTGNPTVIK.M 1332.1 1 21/34

5523, 1-2 K.VDQVQDIVTGNPTVIK.M 2271.6 1 24/30

5648, 1 K.VDQVQDIVTGNPTVIK.M 2151.8 1 24/30

5670, 2-2 R.ITLEDVISHSK.K 1916.5 1 17/20

Page 53: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5793, 1 K.TLVGSENPPLTVIR.K 1045.3 1 20/26

5822, 1-2 R.ITLEDVISHSK.K 1532.7 1 16/20

6182, 1 K.LPYDVTTEQALTYPEVK.N 1606.9 1 23/32

6777, 2-2 K.AAFYEEQINYYDTYIK.T 1060.2 1 20/30

6875, 2-2 R.VVAVGYINEAIDEGNPLR.T 1555.6 1 20/34

6993, 1-2 K.AAFYEEQINYYDTYIK.T 793.7 1 16/30

7085, 1-2 K.GVLLDIDDLQTNQFK.N 1372.8 1 18/28

7092, 2-2 R.NPNAVLTLVDDNLAPEYQK.E 618.0 1 20/36

7322, 1-2 R.NPNAVLTLVDDNLAPEYQK.E 756.1 1 20/36

7366, 1 K.QWVTLVVDVNQCLEGKK.S 660.3 1 16/32

7462, 1 R.NPNAVLTLVDDNLAPEYQK.E 1370.6 1 25/36

7872, 2-2 R.ENIWSASEELLLR.F 1372.3 1 18/24

8128, 1-2 R.ENIWSASEELLLR.F 957.8 1 16/24

8247, 1 R.ENIWSASEELLLR.F 1503.2 1 19/24

8694, 1 K.VNVNLLIYLLNK.K 1824.5 1 18/22

8768, 1-2 K.EIEDIIEEVTVGYIR.E 921.3 1 20/28

8853, 1 K.EIEDIIEEVTVGYIR.E 2156.9 1 23/28

9086, 1 K.VLNSIISSLDLLPYGLR.Y 613.7 1 17/32

10059, 1 K.IGGILANELSVDEAALHAAVIAINEAVEK.G

973.3 1 35/112

6 spectrin beta chain, brain 1 isoform 1 274606.5

11238225

0

23 (23

0 0 0 0)

4368, 1-2 K.TALPAQSAATLPAR.T 707.5 1 16/26

4492, 2-2 K.KHEAIETDIAAYEER.V 1119.3 1 19/28

4666, 1 K.IVSSSDVGHDEYSTQSLVK.K 1668.7 1 25/36

5001, 1 R.KKEIEELQSQAQALSQEGK.S 2000.5 1 33/72

5192, 1-2 R.LILEVHQFSR.D 976.5 1 14/18

5194, 1-2 K.EIEELQSQAQALSQEGK.S 1027.6 1 19/32

5204, 2-2 R.SQNIVTDSSSLSAEAIR.Q 2618.6 1 24/32

5266, 2-2 R.LQALDTGWNELHK.M 1705.0 1 19/24

5339, 1 K.EIEELQSQAQALSQEGK.S 1563.2 1 23/32

5384, 1 R.FQIQDISVETEDNKEK.K 1651.0 1 22/30

5495, 1 R.SQNIVTDSSSLSAEAIR.Q 2004.5 1 25/32

6814, 2-2 R.EVDDLEQWIAER.E 1108.5 1 15/22

6868, 1-2 R.LTTLELLEVR.R 1022.2 1 15/18

6980, 1 R.LTTLELLEVR.R 1480.0 1 17/18

7047, 1 K.DALLSALSIQNYHLECNETK.S 963.4 1 17/38

7094, 2-2 R.LVSQDNFGFDLPAVEAATK.K 566.6 1 18/36

7433, 1-2 K.FANSLVGVQQQLQAFNTYR.T 1458.2 1 21/36

7468, 1-2 R.DLDDFQSWLSR.T 1587.4 1 17/20

7558, 2-2 R.DASVAEAWLLGQEPYLSSR.E 597.0 1 15/36

7618, 1 R.DLDDFQSWLSR.T 1457.9 1 17/20

7799, 1-2 R.DASVAEAWLLGQEPYLSSR.E 1030.0 1 21/36

7955, 1-2 K.GNLEVLLFTIQSK.M 582.5 1 12/24

9569, 1 R.LWEYLLELLR.A 1699.7 1 16/18

7 myosin-9 226529.8 12667788

21 (20

1 0 0 0)

4571, 1-2 R.QAQQERDELADEIANSSGK.G 1057.7 1 27/72

4692, 2-2 R.IAEFTTNLTEEEEKSK.S 813.4 1 16/30

4719, 1-2 K.IAQLEEQLDNETKER.Q 1177.1 1 20/28

4809, 1-2 R.IAEFTTNLTEEEEKSK.S 1376.5 1 19/30

Page 54: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

4858, 1-2 K.VEAQLQELQVK.F 1071.2 1 14/20

5081, 1-2 K.KQELEEICHDLEAR.V 1795.8 1 19/26

5249, 2-2 K.ALELDSNLYR.I 1338.9 1 16/18

5480, 2-2 K.VIQYLAYVASSHK.S 2109.1 1 20/24

5520, 1-2 K.TDLLLEPYNK.Y 1172.7 1 15/18

5628, 1 K.HSQAVEELAEQLEQTKR.V 1104.8 1 28/64

6172, 1 K.EQADFAIEALAK.A 617.9 1 14/22

6423, 1-2 K.VSHLLGINVTDFTR.G 1591.7 1 20/26

7176, 1 K.LQVELDNVTGLLSQSDSK.S 1189.5 1 22/34

7194, 1 R.ELESQISELQEDLESER.A 1568.4 1 24/32

7479, 1-2 R.LQQELDDLLVDLDHQR.Q 1379.6 1 19/30

7601, 1 R.LQQELDDLLVDLDHQR.Q 1185.9 1 19/30

8030, 1-2 R.VISGVLQLGNIVFK.K 1793.5 1 20/26

8034, 1-2 K.DFSALESQLQDTQELLQEENR.Q 1023.6 1 20/40

8145, 1 R.VISGVLQLGNIVFK.K 2101.0 1 21/26

8178, 1 K.DFSALESQLQDTQELLQEENR.Q 1116.5 1 22/40

8940, 1 R.INFDVNGYIVGANIETYLLEK.S 657.3 1 18/40

8 plectin isoform 1e 513705.9 41322908

19 (18

1 0 0 0)

4401, 1-2 R.SSSVGSSSSYPISPAVSR.T 532.6 1 20/34

4536, 1-2 R.VPLDEALQR.G 524.0 1 14/16

4661, 1 K.AYSDPSTGEPATYGELQQR.C 841.1 1 22/36

5393, 1-2 K.GFFDPNTHENLTYR.Q 803.3 1 17/26

5556, 2-2 K.AKLEQLFQDEVAK.A 1369.1 1 17/24

5619, 1-2 R.LQEAGILSAEELQR.L 1813.1 1 21/26

5651, 1-2 R.LLDAQLSTGGIVDPSK.S 972.8 1 18/30

5981, 2-2 R.LTAEDLFEAR.I 1390.7 1 16/18

5988, 1-2 R.SLESLHSFVAAATK.E 842.2 1 16/26

6130, 1-2 R.LTAEDLFEAR.I 975.0 1 15/18

6292, 2-2 R.SLVPAAELLESR.V 786.1 1 18/22

6435, 2-2 K.VQSGSESVIQEYVDLR.T 850.1 1 16/30

6449, 1-2 R.SLVPAAELLESR.V 632.5 1 17/22

6642, 1 R.QTNLENLDQAFSVAER.D 671.9 2 16/30

6677, 1-2 R.APVPASELLASGVLSR.A 1422.7 1 22/30

7042, 1 K.LQNVQIALDYLR.H 1180.3 1 17/22

7290, 1-2 R.ESADPLGAWLQDAR.R 1039.4 1 18/26

7406, 2-2 R.GLFDEEMNEILTDPSDDTK.G 567.9 1 14/36

7468, 1 K.FLEGTSCIAGVFVDATK.E 859.3 1 19/32

9 alcohol dehydrogenase 4 40221.5 71565152

37 (37

0 0 0 0)

3873, 2-2 K.ALGATDCLNPR.D 1962.0 1 19/20

3939, 1-2 K.ALGATDCLNPR.D 1790.1 1 18/20

3972, 1-2 K.ALGATDCLNPR.D 1308.2 1 16/20

4644, 1-2 K.ISEAFDLM*NQGK.S 660.6 1 14/22

4947, 2-2 K.GGVDFALDCAGGSETM*K.A 2048.5 1 23/32

4986, 2-2 R.IIGIDINSEK.F 685.9 1 12/18

4998, 2-2 R.IIGIDINSEK.F 690.1 1 12/18

5109, 1-2 R.IIGIDINSEK.F 1011.5 1 13/18

5256, 1 K.GGVDFALDCAGGSETM*K.A 654.4 1 20/32

5482, 1-2 K.GGVDFALDCAGGSETMK.A 1680.7 1 23/32

5548, 2-2 R.IQIIATSLCHTDATVIDSK.F 3189.2 1 26/36

Page 55: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5690, 1-2 R.IQIIATSLCHTDATVIDSK.F 2432.5 1 25/36

5970, 1-2 K.CKFCLSPLTNLCGK.I 634.5 1 14/26

6240, 2-2 R.VCLLGCGFSTGYGAAINNAK.V 3468.9 1 29/38

6257, 2-2 R.VCLLGCGFSTGYGAAINNAK.V 3328.6 1 29/38

6335, 2-2 K.AAIAWEAGKPLCIEEVEVAPPK.A 957.4 1 24/42

6350, 2-2 K.FCLSPLTNLCGK.I 781.2 1 17/22

6440, 1-2 R.VCLLGCGFSTGYGAAINNAK.V 3546.2 1 29/38

6501, 2-2 K.AALDCTTAGWGSCTFIGVAAGSK.G

1384.6 1 25/44

6524, 1 R.VCLLGCGFSTGYGAAINNAK.V 1664.6 1 34/76

6536, 1-2 K.AAIAWEAGKPLCIEEVEVAPPK.A 1290.4 1 28/42

6612, 1 K.AAIAWEAGKPLCIEEVEVAPPK.A 1146.5 1 26/42

6620, 1 K.FCLSPLTNLCGK.I 697.1 1 16/22

6648, 1 R.VCLLGCGFSTGYGAAINNAK.V 1708.7 1 26/38

6689, 1-2 K.AALDCTTAGWGSCTFIGVAAGSK.G

1169.6 1 23/44

7034, 2-2 R.DLHKPIQEVIIELTK.G 927.4 1 20/28

7041, 2-2 R.DLHKPIQEVIIELTK.G 1081.9 1 21/28

7167, 1-2 K.KFNLDALVTHTLPFDK.I 912.4 1 16/30

7198, 1 K.GKPVYHFFGTSTFSQYTVVSDINLAK.I

1137.9 1 33/100

7247, 1 K.KFNLDALVTHTLPFDK.I 977.0 1 17/30

7262, 1-2 R.DLHKPIQEVIIELTK.G 1055.7 1 21/28

7275, 1-2 R.DLHKPIQEVIIELTK.G 996.1 1 21/28

7335, 1 R.DLHKPIQEVIIELTK.G 571.2 1 16/28

7378, 1 R.DLHKPIQEVIIELTK.G 2105.3 1 32/56

7407, 1 R.DLHKPIQEVIIELTK.G 2061.1 1 31/56

7786, 1-2 K.FNLDALVTHTLPFDK.I 1582.6 1 21/28

9273, 1 K.FNLDALVTHTLPFDKISEAFDLMNQGK.S

1054.5 1 32/104

10 spectrin alpha chain, brain isoform 1 285091.3

19459550

9

22 (20

2 0 0 0)

3729, 1-2 K.LSDDNTIGKEEIQQR.L 949.4 1 19/28

4430, 1-2 K.LIQEQHPEEELIK.T 589.8 4 13/24

4871, 1-2 R.DLAALEDKVK.A 1519.6 1 15/18

5162, 1 K.ALCAEADRLQQSHPLSATQIQVK.R 1327.1 1 33/88

5663, 1-2 R.DLAALGDKVNSLGETAER.L 655.3 1 17/34

5852, 1 K.CTELNQAWSSLGK.R 748.7 1 16/24

6153, 1-2 R.EANELQQWINEK.E 967.5 1 13/22

6628, 2-2 R.SSLSSAQADFNQLAELDR.Q 1064.5 2 19/34

6843, 1-2 R.SSLSSAQADFNQLAELDR.Q 2109.0 1 24/34

6846, 1 R.KVEDLFLTFAK.K 1260.8 1 17/20

7172, 1-2 R.AQLADSFHLQQFFR.D 826.2 1 16/26

7252, 1-2 K.LDENSAFLQFNWK.A 711.0 1 14/24

7252, 2-2 R.LAALADQWQFLVQK.S 818.0 1 14/26

7470, 1-2 R.LAALADQWQFLVQK.S 1603.6 1 18/26

8030, 1 R.GLVSSDELAKDVTGAEALLER.H 608.3 1 15/40

8078, 2-2 K.IAALQAFADQLIAAGHYAK.G 1197.5 1 33/72

8162, 1 R.AGTFQAFEQFGQQLLAHGHYASPEIK.Q

612.5 1 27/100

8355, 1-2 K.IAALQAFADQLIAAGHYAK.G 1644.2 1 20/36

Page 56: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

8422, 1 K.IAALQAFADQLIAAGHYAK.G 1610.2 1 34/72

8438, 1 K.IAALQAFADQLIAAGHYAK.G 1683.1 1 21/36

9080, 1 K.ALINADELASDVAGAEALLDR.H 1575.7 1 30/80

9081, 1 K.ALINADELASDVAGAEALLDR.H 1591.2 1 22/40

11 epoxide hydrolase 1 precursor 52948.5

20986283

7

31 (31

0 0 0 0)

3909, 2-2 R.FYKENLGQGWM*TQKHER.M 1784.5 1 29/64

4596, 2-2 R.EDDSIRPFKVETSDEEIHDLHQR.I 670.0 1 34/88

4607, 1-2 R.FYKENLGQGWM*TQK.H 545.3 1 16/26

4760, 1-2 R.EDDSIRPFKVETSDEEIHDLHQR.I 1113.8 1 28/88

4900, 1 R.EDDSIRPFKVETSDEEIHDLHQR.I 1333.9 1 32/88

5714, 2-2 R.DVELLYPVK.E 994.2 1 12/16

5728, 2-2 R.DVELLYPVK.E 1015.4 1 12/16

5865, 1-2 R.DVELLYPVK.E 948.5 1 12/16

5879, 1-2 R.DVELLYPVK.E 1102.2 1 12/16

6212, 2-2 R.GGHFAAFEEPELLAQDIRK.F 1565.2 1 33/72

6264, 2-2 K.NHGLSDEHVFEVICPSIPGYGFSEASSKK.G

690.0 1 32/112

6447, 1-2 R.GGHFAAFEEPELLAQDIRK.F 2061.2 1 38/72

6476, 1 R.GGHFAAFEEPELLAQDIRK.F 2044.0 1 26/36

6554, 2-2 K.NHGLSDEHVFEVICPSIPGYGFSEASSK.K

934.1 1 37/108

6686, 2-2 R.DKEETLPLEDGWWGPGTR.S 733.5 1 22/34

6711, 2-2 R.GGHFAAFEEPELLAQDIR.K 1934.0 1 24/34

6729, 2-2 R.GGHFAAFEEPELLAQDIR.K 2449.3 1 27/34

6876, 1 K.NHGLSDEHVFEVICPSIPGYGFSEASSK.K

581.5 1 18/54

6940, 1-2 R.GGHFAAFEEPELLAQDIR.K 2144.6 1 25/34

6962, 1-2 R.GGHFAAFEEPELLAQDIR.K 2476.3 1 27/34

7013, 1 R.DKEETLPLEDGWWGPGTR.S 677.8 1 22/34

7539, 1-2 R.FLGLTERDVELLYPVK.E 533.2 1 16/30

7614, 2-2 K.PLLMVHGWPGSFYEFYK.I 1050.2 1 20/32

7665, 2-2 R.ESGYM*HIQCTKPDTVGSALNDSPVGLAAYILEK.F

823.6 1 33/128

7797, 2-2 R.ESGYMHIQCTKPDTVGSALNDSPVGLAAYILEK.F

835.7 1 31/128

7881, 1-2 K.PLLMVHGWPGSFYEFYK.I 930.0 1 19/32

7966, 1 K.PLLMVHGWPGSFYEFYK.I 807.7 1 18/32

8043, 1 R.ESGYM*HIQCTKPDTVGSALNDSPVGLAAYILEK.F

1183.2 1 38/128

8057, 1-2 R.ESGYMHIQCTKPDTVGSALNDSPVGLAAYILEK.F

969.1 1 35/128

8361, 1-2 K.VYVPTGFSAFPFELLHTPEK.W 527.6 1 19/38

8450, 1 K.VYVPTGFSAFPFELLHTPEK.W 550.0 1 19/38

12 17-beta-hydroxysteroid dehydrogenase 13 isoform B 29604.7

21003211

2

46 (46

0 0 0 0)

5127, 1-2 R.MQNIQFEAVVGHK.I 852.6 2 15/24

5282, 1 R.MQNIQFEAVVGHK.I 1232.8 1 17/24

5283, 1 R.MQNIQFEAVVGHK.I 1298.1 1 26/48

5534, 1-2 R.SLIDGILTNKK.M 786.6 1 14/20

5672, 2-2 R.KSVAGEIVLITGAGHGIGR.Q 1581.6 1 34/72

Page 57: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5678, 2-2 R.KSVAGEIVLITGAGHGIGR.Q 2331.2 1 26/36

5775, 2-2 R.NHGHIVTVASVCGHEGIPYLIPYCSSK.F

744.4 1 30/104

5802, 1-2 R.KSVAGEIVLITGAGHGIGR.Q 1226.8 1 32/72

5810, 1-2 R.GLTSELQALGK.T 1353.9 1 17/20

5864, 1 R.KSVAGEIVLITGAGHGIGR.Q 1629.8 1 33/72

5872, 1 R.KSVAGEIVLITGAGHGIGR.Q 2672.0 1 28/36

5882, 1 R.GLTSELQALGK.T 1233.1 1 17/20

5930, 1-2 R.NHGHIVTVASVCGHEGIPYLIPYCSSK.F

731.3 1 30/104

5960, 1 R.NHGHIVTVASVCGHEGIPYLIPYCSSK.F

1182.2 1 35/104

6020, 2-2 K.TSCLCPVFVNTGFTK.N 763.7 1 20/28

6093, 2-2 R.NHGHIVTVASVCGHEGIPYLIPYCSSK.F

621.6 1 27/104

6161, 1-2 K.TSCLCPVFVNTGFTK.N 648.7 1 16/28

6566, 1-2 K.KEVGDVTIVVNNAGTVYPADLLSTKDEEITK.T

1723.4 1 42/120

6566, 2-2 K.SVAGEIVLITGAGHGIGR.Q 870.5 1 20/34

6680, 1-2 K.SVAGEIVLITGAGHGIGR.Q 1430.0 1 22/34

6681, 2-2 R.LWPVLETDEVVR.S 524.2 1 15/22

6722, 1 K.KEVGDVTIVVNNAGTVYPADLLSTKDEEITK.T

1745.4 1 40/120

6740, 2-2 R.LWPVLETDEVVR.S 656.6 1 17/22

6758, 1 K.SVAGEIVLITGAGHGIGR.Q 1477.0 1 23/34

6825, 1 K.SVAGEIVLITGAGHGIGR.Q 1544.6 1 24/34

6862, 1 K.KEVGDVTIVVNNAGTVYPADLLSTK.D

1635.5 1 24/48

6927, 1-2 K.SVAGEIVLITGAGHGIGR.Q 708.5 1 17/34

7035, 1 R.LWPVLETDEVVR.S 582.2 1 14/22

7041, 1 R.LWPVLETDEVVR.S 957.6 1 19/22

7086, 2-2 K.EVGDVTIVVNNAGTVYPADLLSTK.D

523.1 1 16/46

7232, 1 K.EVGDVTIVVNNAGTVYPADLLSTKDEEITK.T

1154.0 1 34/116

7326, 2-2 R.QSILVLWDINK.V 505.1 1 13/20

7335, 1-2 K.EVGDVTIVVNNAGTVYPADLLSTK.D

1197.2 1 25/46

7438, 1 K.EVGDVTIVVNNAGTVYPADLLSTK.D

1264.6 1 24/46

7455, 1 K.EVGDVTIVVNNAGTVYPADLLSTK.D

754.9 1 21/46

7703, 1 R.QSILVLWDINK.V 803.5 1 16/20

7985, 1-2 K.TFEVNILGHFWITK.A 1671.2 1 20/26

8076, 1 K.TFEVNILGHFWITK.A 1575.4 1 21/26

8121, 1 K.TFEVNILGHFWITK.A 1148.4 1 19/26

9753, 1 R.LWPVLETDEVVR.S 749.4 1 17/22

9984, 1 K.TFEVNILGHFWITK.A 1026.8 1 16/26

10324, 1 K.TFEVNILGHFWITK.A 649.3 1 16/26

10659, 1 K.TFEVNILGHFWITK.A 660.8 2 14/26

Page 58: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

11326, 1 K.TFEVNILGHFWITK.A 526.4 1 12/26

12052, 1 K.SVAGEIVLITGAGHGIGR.Q 731.3 1 16/34

12080, 1 K.TSCLCPVFVNTGFTK.N 656.5 1 18/28

13 carbamoyl-phosphate synthase [ammonia], mitochondrial isoform c 116037.4

17029579

7

23 (23

0 0 0 0)

5406, 2-2 R.SAYALGGLGSGICPNR.E 1216.0 1 20/30

5408, 2-2 K.TVLM*NPNIASVQTNEVGLK.Q 1009.5 1 19/36

5675, 1-2 R.FLGVAEQLHNEGFK.L 1729.5 1 21/26

5694, 1 K.TVLM*NPNIASVQTNEVGLK.Q 836.3 1 18/36

5856, 2-2 K.VLGTSVESIMATEDR.Q 997.5 1 16/28

5876, 1 K.AFAMTNQILVEK.S 1551.6 1 18/22

5915, 1 K.AADTIGYPVMIR.S 525.3 1 14/22

6006, 1-2 K.VLGTSVESIMATEDR.Q 1572.3 1 20/28

6094, 1 K.VLGTSVESIMATEDR.Q 974.4 1 18/28

6144, 2-2 K.HLPTLDHPIIPADYVAIK.A 610.3 1 27/68

6368, 1 K.HLPTLDHPIIPADYVAIK.A 916.2 1 31/68

6387, 1 K.HLPTLDHPIIPADYVAIK.A 756.6 1 18/34

6459, 2-2 K.TLGVDFIDVATK.V 910.7 1 18/22

6651, 1-2 K.TLGVDFIDVATK.V 808.1 1 17/22

6747, 1 K.TLGVDFIDVATK.V 915.4 1 18/22

7049, 2-2 K.IALGIPLPEIK.N 526.3 1 16/20

7270, 1-2 K.IALGIPLPEIK.N 567.8 1 16/20

7440, 1-2 R.TAVDSGIPLLTNFQVTK.L 576.7 1 21/32

7524, 1 R.HLGIVGECNIQFALHPTSMEYCIIEVNAR.L

875.1 1 31/112

7550, 1 R.TAVDSGIPLLTNFQVTK.L 746.6 1 22/32

7960, 1-2 K.IAPSFAVESIEDALK.A 1345.0 1 22/28

8246, 1-2 K.AFAISGPFNVQFLVK.G 937.7 1 19/28

8721, 1 R.SIFSAVLDELK.V 934.3 1 15/20

14 ATP synthase subunit beta, mitochondrial precursor 56559.5 32189394

21 (21

0 0 0 0)

4662, 2-2 K.AHGGYSVFAGVGER.T 1832.6 1 23/26

4769, 1 R.IM*DPNIVGSEHYDVAR.G 638.8 1 18/30

5037, 1-2 K.VALVYGQMNEPPGAR.A 566.9 1 15/28

5346, 2-2 R.IMNVIGEPIDER.G 928.5 1 15/22

5500, 1-2 R.IMNVIGEPIDER.G 1209.7 1 17/22

5594, 2-2 R.LVLEVAQHLGESTVR.T 1730.9 1 21/28

5729, 1-2 R.LVLEVAQHLGESTVR.T 1105.2 1 17/28

5847, 1 R.LVLEVAQHLGESTVR.T 1487.3 1 20/28

6129, 1-2 K.VLDSGAPIKIPVGPETLGR.I 982.3 1 29/72

7220, 2-2 R.VALTGLTVAEYFR.D 1397.6 1 18/24

7233, 1 R.AIAELGIYPAVDPLDSTSR.I 543.9 1 19/36

7464, 1-2 R.VALTGLTVAEYFR.D 1636.8 1 19/24

7551, 1 K.KGSITSVQAIYVPADDLTDPAPATTFAHLDATTVLSR.A

1884.0 1 46/144

7616, 1 R.VALTGLTVAEYFR.D 1472.8 1 19/24

7712, 1-2 R.FLSQPFQVAEVFTGHMGK.L 682.2 1 29/68

7780, 1-2 K.TVLIM*ELINNVAK.A 1055.7 1 15/24

7958, 1 K.GSITSVQAIYVPADDLTDPAPATTFAHLDATTVLSR.A

863.3 1 36/140

8481, 2-2 K.TVLIMELINNVAK.A 1697.3 1 18/24

Page 59: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

8774, 1-2 K.TVLIMELINNVAK.A 2176.9 1 19/24

8834, 1 K.TVLIMELINNVAK.A 2180.6 1 19/24

9166, 1 R.LVLEVAQHLGESTVR.T 947.6 1 16/28

15 peroxisomal multifunctional enzyme type 2 isoform 1 83024.9

31348281

0

21 (21

0 0 0 0)

4068, 1-2 R.RVLQQFADNDVSR.F 795.1 1 17/24

4302, 1-2 R.VLQQFADNDVSR.F 1341.0 1 18/22

4412, 1-2 R.ATSTATSGFAGAIGQK.L 2067.5 1 23/30

4433, 1-2 R.ATSTATSGFAGAIGQK.L 1681.0 1 21/30

4976, 1-2 K.GSLAADKVVEEIR.R 1281.3 1 16/24

5296, 2-2 R.ISDEDWDIIHR.V 1535.8 1 16/20

5558, 1 R.ISDEDWDIIHR.V 1445.8 1 16/20

5592, 1-2 K.VLHGEQYLELYKPLPR.A 1849.7 1 21/30

5645, 1-2 R.IDVVVNNAGILR.D 1041.6 1 16/22

5692, 1 K.VLHGEQYLELYKPLPR.A 1603.1 1 20/30

5751, 1 R.IDVVVNNAGILR.D 1336.2 1 17/22

5867, 2-2 R.IIMTSSASGIYGNFGQANYSAAK.L 612.9 1 19/44

6293, 2-2 K.VQETGDIVISNAYVDLAPTSGTSAK.T

1230.7 1 23/48

6368, 2-2 K.VAVAIPNRPPDAVLTDTTSLNQAALYR.L

1965.9 1 39/104

6478, 1-2 K.VQETGDIVISNAYVDLAPTSGTSAK.T

1120.8 1 24/48

6545, 1-2 K.VAVAIPNRPPDAVLTDTTSLNQAALYR.L

1029.2 1 32/104

6779, 1 K.LQSTFVFEEIGR.R 918.4 1 15/22

7658, 2-2 K.LGLLGLANSLAIEGR.K 1557.5 1 21/28

8013, 1 K.LGLLGLANSLAIEGR.K 879.5 1 17/28

8247, 1-2 K.FIYEGSSDFSCLPTFGVIIGQK.S 879.6 1 23/42

8367, 1 K.FIYEGSSDFSCLPTFGVIIGQK.S 814.0 1 20/42

16 tubulin beta-2A chain 49906.7 4507729

26 (20

6 0 0 0)

3852, 1-2 -.M*REIVHIQAGQCGNQIGAK.F 791.6 1 22/72

4022, 2-2 R.EIVHIQAGQCGNQIGAK.F 2424.0 1 25/32

4023, 2-2 R.EIVHIQAGQCGNQIGAK.F 2023.2 1 31/64

4104, 1-2 R.EIVHIQAGQCGNQIGAK.F 1975.4 1 30/64

4134, 1-2 R.EIVHIQAGQCGNQIGAK.F 1002.4 1 22/32

5676, 1-2 R.KLAVNMVPFPR.L 824.7 1 15/20

5866, 1-2 R.AILVDLEPGTM*DSVR.S 628.8 1 18/28

5974, 1 R.AILVDLEPGTM*DSVR.S 791.6 2 18/28

6110, 2-2 K.LAVNMVPFPR.L 869.2 1 17/18

6281, 1-2 K.LAVNMVPFPR.L 885.5 1 17/18

6309, 2-2 R.AILVDLEPGTMDSVR.S 710.3 1 19/28

6830, 2-2 K.NSSYFVEWIPNNVK.T 605.7 1 15/26

7036, 1-2 K.NSSYFVEWIPNNVK.T 569.2 1 15/26

7203, 2-2 K.GHYTEGAELVDSVLDVVRK.E 659.9 1 26/72

7262, 2-2 R.LHFFMPGFAPLTSR.G 654.7 1 16/26

7403, 2-2 K.NSSYFVEWIPNNVK.T 517.6 1 14/26

7450, 2-2 K.M*SATFIGNSTAIQELFK.R 770.3 1 17/32

7484, 1-2 R.LHFFMPGFAPLTSR.G 582.7 1 16/26

7534, 1 K.GHYTEGAELVDSVLDVVRK.E 1522.4 1 34/72

Page 60: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7587, 1 R.LHFFMPGFAPLTSR.G 576.7 1 16/26

7613, 2-2 K.GHYTEGAELVDSVLDVVR.K 2982.8 1 26/34

7630, 1-2 R.SGPFGQIFRPDNFVFGQSGAGNNWAK.G

627.8 1 25/100

7792, 1 R.SGPFGQIFRPDNFVFGQSGAGNNWAK.G

704.2 1 20/50

8260, 1-2 K.LTTPTYGDLNHLVSATMSGVTTCLR.F

579.7 1 18/48

8270, 1-2 K.LTTPTYGDLNHLVSATMSGVTTCLR.F

1310.8 1 33/96

8355, 1 K.LTTPTYGDLNHLVSATMSGVTTCLR.F

883.4 1 28/96

17 bile acyl-CoA synthetase precursor 75384.7 13325057

18 (18

0 0 0 0)

4188, 2-2 R.YLCNIPQQPEDR.T 644.1 1 17/22

4275, 1-2 R.YLCNIPQQPEDR.T 534.1 1 16/22

4448, 2-2 R.GMPLAHSVLSSGAR.V 800.3 1 17/26

4956, 1-2 K.VGM*AAVQLAPGQTFDGEK.L 656.1 1 19/34

5492, 2-2 K.VGMAAVQLAPGQTFDGEK.L 726.5 1 20/34

5643, 1-2 K.VGMAAVQLAPGQTFDGEK.L 805.6 1 21/34

6429, 2-2 R.SPALFIYTSGTTGLPK.P 1294.9 1 17/30

6598, 2-2 R.CFYLSHTSPTPGVGALGAALDAAPSHPVPADLR.A

1616.6 1 39/128

6740, 1-2 R.SPALFIYTSGTTGLPKPAILTHER.V 559.4 1 27/92

6897, 1 R.CFYLSHTSPTPGVGALGAALDAAPSHPVPADLR.A

1943.7 1 40/128

7578, 2-2 R.ALLVWTGPGAGSVTFGELDAR.A 1841.8 1 28/40

7834, 1-2 R.ALLVWTGPGAGSVTFGELDAR.A 2212.9 1 29/40

7976, 1 R.ALLVWTGPGAGSVTFGELDAR.A 2142.3 1 26/40

8105, 1 R.WKGENVSTHEVEGVLSQVDFLQQVNVYGVCVPGCEGK.V

1383.0 1 37/144

8261, 1 R.DNQGFCIPVGLGEPGLLLTK.V 547.7 1 18/38

8262, 1 K.GENVSTHEVEGVLSQVDFLQQVNVYGVCVPGCEGK.V

747.0 1 31/136

8865, 2-2 R.EGFNVGIVVDPLFVLDNR.A 732.8 1 18/34

9143, 1-2 R.EGFNVGIVVDPLFVLDNR.A 542.3 1 15/34

18 ATP synthase subunit alpha, mitochondrial precursor 59750.3 4757810

18 (18

0 0 0 0)

4646, 2-2 R.VVDALGNAIDGK.G 1143.4 1 18/22

4944, 2-2 R.VVDALGNAIDGKGPIGSK.T 895.0 1 19/34

5144, 2-2 K.HALIIYDDLSK.Q 1585.0 1 18/20

5188, 1-2 R.ILGADTSVDLEETGR.V 2067.0 1 22/28

5274, 1-2 K.HALIIYDDLSK.Q 1649.9 1 18/20

5304, 2-2 K.TGTAEMSSILEER.I 943.0 1 15/24

5577, 1-2 R.EAYPGDVFYLHSR.L 1051.7 1 18/24

5696, 1 R.EAYPGDVFYLHSR.L 974.3 1 17/24

5830, 1-2 K.TSIAIDTIINQK.R 1864.5 1 18/22

5924, 1 K.TSIAIDTIINQK.R 1434.5 1 17/22

6665, 2-2 R.TGAIVDVPVGEELLGR.V 779.1 1 20/30

7024, 1 R.TGAIVDVPVGEELLGR.V 856.8 1 20/30

7095, 1 K.FENAFLSHVVSQHQALLGTIR.A 1549.8 1 31/80

Page 61: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7108, 1 K.FENAFLSHVVSQHQALLGTIR.A 1694.0 1 24/40

7428, 1-2 K.GMSLNLEPDNVGVVVFGNDK.L 531.0 1 18/38

7858, 2-2 K.LKEIVTNFLAGFEA.- 773.6 1 15/26

9128, 1 R.EVAAFAQFGSDLDAATQQLLSR.G 1035.8 1 29/84

9154, 1 R.EVAAFAQFGSDLDAATQQLLSR.G 872.1 1 21/42

19 probable saccharopine dehydrogenase 47151.1 55770836

18 (18

0 0 0 0)

4610, 1-2 K.SAIYGFGDQSNLRK.L 908.5 1 17/26

4880, 2-2 R.NVSNLKPVPLIGPK.L 791.2 1 18/26

4976, 2-2 K.QATVVLNCVGPYR.F 1288.0 1 18/24

5007, 2-2 R.LPWAVAGR.S 692.2 1 10/14

5014, 1-2 R.NVSNLKPVPLIGPK.L 871.8 1 19/26

5102, 1-2 K.QATVVLNCVGPYR.F 1323.4 1 18/24

5129, 2-2 K.AGGVFTPGAAFSK.T 976.2 1 19/24

5265, 1-2 K.AGGVFTPGAAFSK.T 914.7 1 18/24

5266, 1-2 K.SAIYGFGDQSNLR.K 1284.7 1 19/24

8094, 1 K.AADKGVYIIGSSGFDSIPADLGVIYTR.N

910.7 1 23/52

8242, 1 K.QIDAASFTLTFFGQGYSQGTGTDK.N

888.1 1 20/46

8248, 1 K.QIDAASFTLTFFGQGYSQGTGTDK.N

1314.2 1 32/92

8454, 1-2 K.GVYIIGSSGFDSIPADLGVIYTR.N 584.4 1 22/44

8558, 1 K.MNGTLTAVESFLTIHSGPEGLSIHDGTWK.S

681.4 1 28/112

9453, 1 R.YLYENLEESPVQYAAYVTVGGITSVIK.L

677.3 1 18/52

9456, 1 R.YLYENLEESPVQYAAYVTVGGITSVIK.L

1288.1 1 36/104

10226, 1 K.GPEAGYVATPIAMVQAAMTLLSDASHLPK.A

855.3 1 32/112

10240, 1 K.GPEAGYVATPIAMVQAAMTLLSDASHLPK.A

958.6 1 21/56

20 trifunctional enzyme subunit alpha, mitochondrial precursor 82999.1 20127408

16 (16

0 0 0 0)

4402, 2-2 K.SEVSSDEDIQFR.L 1183.4 1 17/22

4877, 2-2 K.TLQEVTQLSQEAQR.I 1622.9 1 19/26

5004, 1-2 K.TLQEVTQLSQEAQR.I 615.5 2 19/26

5187, 1 K.TLQEVTQLSQEAQR.I 1534.0 1 18/26

5235, 2-2 K.GFYIYQEGVK.R 962.2 1 15/18

5292, 1-2 K.ALMGLYHGQVLCK.K 1297.8 1 16/24

6099, 2-2 R.FGGGNPELLTQMVSK.G 749.8 1 20/28

6273, 1-2 R.FGGGNPELLTQMVSK.G 925.1 1 21/28

7082, 2-2 K.LTAYAMTIPFVR.Q 931.5 1 15/22

7256, 1 K.MQLLEIITTEK.T 1547.6 1 18/20

7289, 2-2 K.TVLGTPEVLLGALPGAGGTQR.L 540.4 1 20/40

7907, 1 R.DSIFSNLTGQLDYQGFEK.A 2230.9 1 25/34

8457, 1-2 R.TIEYLEEVAITFAK.G 752.4 1 15/26

8582, 1 R.TIEYLEEVAITFAK.G 1236.3 1 18/26

8646, 1-2 K.DLNSDMDSILASLK.L 1954.6 1 18/26

Page 62: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

8721, 1-2 K.ADM*VIEAVFEDLSLK.H 1642.8 1 20/28

21 lanosterol synthase isoform 2 82198.1

22417755

6

16 (16

0 0 0 0)

4094, 1-2 R.NPDGGFATYETK.R 849.9 1 17/22

4143, 2-2 K.SCAISYTSYR.N 1088.9 1 16/18

4216, 1-2 K.SCAISYTSYR.N 1231.7 1 17/18

4319, 2-2 K.LYEHIVADDRFTK.S 1224.7 1 17/24

4430, 1-2 K.LYEHIVADDRFTK.S 1215.2 1 17/24

5000, 2-2 R.IPLPAGYREEIVR.Y 507.7 1 13/24

5541, 2-2 R.WYVDGPASTAFQEHVSR.I 1056.7 1 20/32

5577, 1 R.VVYALLNLYEHHHSAHLR.Q 785.7 1 28/68

5578, 1-2 R.ETLTQGLEFCR.R 603.7 1 13/20

5606, 2-2 R.LSAAEDPLVQSLR.Q 1021.2 1 18/24

5720, 2-2 R.ILGVGPDDPDLVR.A 901.2 1 18/24

5840, 1 R.LSAAEDPLVQSLR.Q 1446.5 1 20/24

6380, 2-2 R.NNVAPDELYTPHSWLLR.V 1263.7 1 22/32

6644, 1 R.NNVAPDELYTPHSWLLR.V 890.1 1 18/32

7539, 1 K.STVFGTALNYVSLR.I 1303.9 1 18/26

8796, 1 K.MQGTNGSQIWDTAFAIQALLEAGGHHRPEFSSCLQK.A

580.0 1 30/140

22 very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2

precursor 68058.1 76496475

15 (15

0 0 0 0)

5512, 2-2 R.ALEQFATVVEAK.L 1342.2 1 16/22

5861, 2-2 K.ASNTAEVFFDGVR.V 1346.9 1 17/24

6176, 1 K.GIVNEQFLLQR.L 1225.0 1 15/20

6191, 1 R.FGMAAALAGTMR.G 1731.3 1 18/22

6304, 1 K.LASGETVAAFCLTEPSSGSDAASIR.T

1559.7 1 34/96

6504, 2-2 K.NPFGNAGLLLGEAGK.Q 1438.3 1 21/28

6778, 1 K.NPFGNAGLLLGEAGK.Q 1076.8 1 19/28

6863, 1 R.AGLGSGLSLSGLVHPELSR.S 1806.5 1 34/72

7857, 1 K.GQLTTDQVFPYPSVLNEEQTQFLK.E

999.7 1 34/92

7863, 1 K.GQLTTDQVFPYPSVLNEEQTQFLK.E

645.3 1 19/46

7934, 1 K.ELGAFGLQVPSELGGVGLCNTQYAR.L

638.3 1 21/48

7954, 1 K.ELGAFGLQVPSELGGVGLCNTQYAR.L

2061.6 1 40/96

9396, 1 R.LADGAIDLYAMVVVLSR.A 1416.8 1 19/32

9512, 1 K.LWISNGGLADIFTVFAK.T 1542.5 1 22/32

9800, 1 K.LWISNGGLADIFTVFAK.T 998.9 1 17/32

23 estradiol 17-beta-dehydrogenase 11 32935.6

14297672

9

21 (21

0 0 0 0)

4498, 1-2 K.VHTFVVDCSNREDIYSSAK.K 1255.9 1 21/36

5472, 1-2 K.NPSTSLGPTLEPEEVVNR.L 703.0 1 18/34

5567, 2-2 K.SKLVLWDINK.H 1290.0 1 15/18

5618, 1 K.NPSTSLGPTLEPEEVVNR.L 1011.4 1 22/34

5704, 1 R.KSVTGEIVLITGAGHGIGR.L 914.1 1 22/36

5717, 1-2 K.SKLVLWDINK.H 1333.0 1 15/18

Page 63: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5720, 1 R.KSVTGEIVLITGAGHGIGR.L 2112.6 1 35/72

5907, 1 K.LVLWDINKHGLEETAAK.C 789.0 1 17/32

6279, 2-2 K.SVTGEIVLITGAGHGIGR.L 791.0 1 19/34

6452, 1-2 K.SVTGEIVLITGAGHGIGR.L 1764.5 1 25/34

6531, 1 K.SVTGEIVLITGAGHGIGR.L 698.7 1 17/34

7070, 2-2 K.TLTDELAALQITGVK.T 2470.5 1 22/28

7305, 1-2 K.TLTDELAALQITGVK.T 2534.5 1 22/28

7883, 1-2 K.SVTGEIVLITGAGHGIGR.L 1712.3 1 22/34

7892, 1 K.TFEVNVLAHFWTTK.A 1959.7 1 21/26

7992, 1 K.SVTGEIVLITGAGHGIGR.L 1257.4 1 21/34

8270, 1 K.VKAEIGDVSILVNNAGVVYTSDLFATQDPQIEK.T

1775.6 1 39/128

8907, 1-2 K.M*IFIPSSIAFLTTLER.I 742.9 1 19/30

8984, 1 K.M*IFIPSSIAFLTTLER.I 1213.6 1 24/30

9059, 2-2 K.MIFIPSSIAFLTTLER.I 943.0 1 22/30

9603, 1 K.TLTDELAALQITGVK.T 1052.3 1 16/28

24 peroxisomal bifunctional enzyme isoform 2 69153.5

26187853

9

17 (17

0 0 0 0)

4514, 1-2 R.TISQDEILER.C 742.9 1 16/18

4527, 2-2 K.VVNSDPVEEAIR.F 945.0 1 18/22

4641, 1-2 K.VVNSDPVEEAIR.F 1079.4 1 19/22

4664, 2-2 R.IPVIAVDSDKNQLATANK.M 1104.6 1 19/34

4743, 2-2 R.KGQGLTGPTLLPGTPAR.K 885.7 1 19/32

4810, 1-2 R.IPVIAVDSDKNQLATANK.M 2237.8 1 26/34

4881, 1-2 R.KGQGLTGPTLLPGTPAR.K 999.5 1 20/32

4894, 1-2 K.GWYQYDKPLGR.I 547.9 1 14/20

5564, 1 K.GQGLTGPTLLPGTPAR.K 615.3 1 18/30

6389, 1 R.VSDLAGLDVGWK.S 1689.3 1 20/22

6396, 1 R.VSDLAGLDVGWK.S 1640.8 1 20/22

7191, 2-2 K.GGPMFYASTVGLPTVLEK.L 1101.4 1 20/34

7247, 2-2 R.YCPIPDVLCELGR.F 804.5 1 18/24

7485, 1-2 R.YCPIPDVLCELGR.F 658.8 1 16/24

7497, 1-2 R.LTGVPAALDLITSGR.R 536.8 1 20/28

7562, 1-2 R.CLYSLINEAFR.I 1557.9 1 17/20

7682, 1 R.CLYSLINEAFR.I 1816.3 1 17/20

25 UDP-glucuronosyltransferase 2B7 precursor 60720.3

19019438

9

16 (16

0 0 0 0)

5213, 2-2 K.IEIYPTSLTK.T 996.7 1 15/18

5936, 1-2 K.ADVWLIR.N 881.4 3 11/12

6485, 2-2 R.ANVIASALAQIPQK.V 650.7 1 15/26

6591, 2-2 K.VLVWAAEYSHWM*NIK.T 614.8 1 16/28

6656, 2-2 K.TILDELIQR.G 704.8 1 14/16

6663, 2-2 K.WDQFYSEVLGRPTTLSETMGK.A 586.1 1 18/40

6681, 1-2 R.ANVIASALAQIPQK.V 856.5 1 17/26

6809, 1-2 K.TELENFIM*QQIK.R 597.6 1 13/22

6884, 2-2 R.VDFNTMSSTDLLNALKR.V 937.7 1 20/32

6978, 1 K.TILDELIQR.G 740.0 1 14/16

7106, 1-2 R.VDFNTMSSTDLLNALKR.V 771.5 1 18/32

7110, 1-2 R.VDFNTMSSTDLLNALKR.V 734.3 1 18/32

7305, 2-2 K.TELENFIMQQIK.R 1416.4 1 16/22

7623, 1-2 R.VDFNTMSSTDLLNALK.R 956.5 1 19/30

Page 64: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7631, 1-2 R.VDFNTMSSTDLLNALK.R 925.9 1 19/30

8902, 1 R.NSWNFQFPYPLLPNVDFVGGLHCK.P

748.0 1 29/92

26 dimethylaniline monooxygenase [N-oxide-forming] 3 60033.2 50541961

21 (21

0 0 0 0)

4911, 2-2 K.HPDFATTGQWDVTTER.D 784.9 1 19/30

5050, 1-2 K.HPDFATTGQWDVTTER.D 688.5 1 18/30

5312, 2-2 R.SCLEEGLEPTCFEK.S 1402.3 1 22/26

5468, 1-2 R.SCLEEGLEPTCFEK.S 1184.5 1 21/26

5616, 2-2 R.NNEIILFK.G 808.9 1 11/14

5620, 2-2 R.NNEIILFK.G 1285.9 1 13/14

5750, 1-2 R.NNEIILFK.G 819.0 1 11/14

6316, 1-2 K.IQEYIIAFAK.E 952.1 1 16/18

6377, 2-2 K.VAIIGAGVSGLASIR.S 1993.8 1 21/28

6626, 2-2 R.VLVVGLGNSGCDIATELSR.T 2091.4 1 26/36

6838, 1-2 R.VLVVGLGNSGCDIATELSR.T 2209.6 1 24/36

6958, 1 R.VLVVGLGNSGCDIATELSR.T 2130.1 1 25/36

7628, 2-2 R.KEPVFNDELPASILCGIVSVKPNVK.E

2069.1 1 34/96

7874, 1-2 R.KEPVFNDELPASILCGIVSVKPNVK.E

1668.4 1 34/96

8007, 1 R.KEPVFNDELPASILCGIVSVKPNVK.E

1253.2 1 30/96

8289, 1-2 K.PNIPWLFLTDPK.L 695.5 1 17/22

8415, 1 K.PNIPWLFLTDPK.L 562.4 1 16/22

8498, 1 K.EPVFNDELPASILCGIVSVKPNVK.E 583.6 1 29/92

8710, 1-2 R.VWDNGYPWDMLLVTR.F 667.5 1 19/28

9564, 1-2 K.VAIIGAGVSGLASIR.S 552.3 1 15/28

11880, 2-2 K.VAIIGAGVSGLASIR.S 538.9 2 13/28

27 perilipin-2 48075.1 34577059

24 (24

0 0 0 0)

5252, 2-2 K.KVEGFDLVQKPSYYVR.L 1633.4 1 23/30

5404, 1-2 K.KVEGFDLVQKPSYYVR.L 1087.3 1 19/30

5468, 2-2 R.LPILNQPSTQIVANAK.G 733.5 1 19/30

5656, 1-2 K.SVVSGSINTVLGSR.M 2331.0 1 21/26

5669, 1-2 K.SVVSGSINTVLGSR.M 2567.1 1 22/26

5676, 1 R.LPILNQPSTQIVANAK.G 781.7 1 19/30

5748, 2-2 K.SQQTISQLHSTVHLIEFAR.K 1756.0 1 36/72

5756, 1 K.SVVSGSINTVLGSR.M 1760.6 1 19/26

5757, 2-2 K.SQQTISQLHSTVHLIEFAR.K 1484.6 1 22/36

5768, 1 K.SVVSGSINTVLGSR.M 1967.8 1 21/26

5900, 1-2 K.SQQTISQLHSTVHLIEFAR.K 1200.6 1 28/72

5927, 1-2 K.SQQTISQLHSTVHLIEFAR.K 1029.7 1 19/36

5972, 1 K.SQQTISQLHSTVHLIEFAR.K 1584.1 1 23/36

6052, 1 K.DSVASTITGVMDK.T 1148.6 1 17/24

6096, 2-2 R.MMQLVSSGVENALTK.S 1538.8 1 22/28

6276, 1-2 R.MMQLVSSGVENALTK.S 1934.4 1 24/28

6358, 1 R.MMQLVSSGVENALTK.S 1743.7 1 22/28

6370, 1 R.MMQLVSSGVENALTK.S 1378.0 1 21/28

6730, 1-2 K.TITSVAMTSALPIIQK.L 917.4 1 18/30

7779, 2-2 K.SELLVEQYLPLTEEELEK.E 989.2 1 22/34

Page 65: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7790, 1 K.LYLSWVEWK.R 844.9 1 14/16

8038, 1-2 K.SELLVEQYLPLTEEELEK.E 1226.1 1 23/34

9093, 1 R.VVNLPLVSSTYDLMSSAYLSTK.D 2009.2 1 26/42

9105, 1 R.VVNLPLVSSTYDLMSSAYLSTK.D 1964.0 1 26/42

28 alpha-actinin-4 104853.3 12025678

17 (17

0 0 0 0)

5426, 1-2 K.LSGSNPYTTVTPQIINSK.W 1217.2 1 20/34

5448, 1-2 R.TINEVENQILTR.D 1345.2 1 17/22

5571, 1 K.LSGSNPYTTVTPQIINSK.W 1655.8 1 23/34

5580, 2-2 R.FAIQDISVEETSAK.E 1093.5 1 19/26

5584, 1 R.TINEVENQILTR.D 1509.9 1 18/22

5883, 1-2 K.QLEAIDQLHLEYAK.R 661.1 1 16/26

6029, 2-2 R.ETTDTDTADQVIASFK.V 1116.4 1 19/30

6084, 1-2 K.ICDQWDALGSLTHSR.R 1391.0 1 20/28

6207, 1-2 R.ETTDTDTADQVIASFK.V 1799.8 1 21/30

6525, 1-2 K.EGLLLWCQR.K 686.2 1 13/16

7606, 2-2 K.AIMTYVSSFYHAFSGAQK.A 674.1 1 15/34

7833, 1-2 K.LASDLLEWIR.R 1074.0 1 15/18

7852, 1-2 K.AIMTYVSSFYHAFSGAQK.A 1138.8 1 20/34

7955, 1 K.AIMTYVSSFYHAFSGAQK.A 1015.4 1 20/34

7956, 1 K.LASDLLEWIR.R 1085.3 1 15/18

8374, 1-2 R.VGWEQLLTTIAR.T 927.2 1 16/22

8456, 1 R.VGWEQLLTTIAR.T 1462.6 1 18/22

29 long-chain-fatty-acid--CoA ligase 5 isoform a 82262.5 42794756

13 (13

0 0 0 0)

4175, 1-2 R.IVQAVVYSCGAR.V 1598.2 1 18/22

4830, 2-2 K.GSFEELCQNQVVR.E 1285.1 1 17/24

4959, 1-2 R.AEYLGSCLLHK.G 515.6 1 13/20

5338, 2-2 R.TQIDSLYEHIQD.- 900.5 1 16/22

5489, 1-2 R.TQIDSLYEHIQD.- 888.6 1 17/22

6489, 1-2 K.GAMITHQNIVSNAAAFLK.C 769.0 1 18/34

6503, 1-2 R.SQPVLQIFVHGESLR.S 903.8 1 20/28

6551, 1 R.SQPVLQIFVHGESLR.S 970.3 1 18/28

7054, 2-2 K.VIILMDPFDDDLKQR.G 543.4 1 17/28

7082, 1 R.HDSFWDKLIFAK.I 625.0 1 14/22

7293, 1-2 K.VIILMDPFDDDLKQR.G 638.7 1 18/28

7647, 1 K.SGIEILSLYDAENLGK.E 966.2 1 19/30

7725, 1 R.SSLVGVVVPDTDVLPSFAAK.L 956.7 1 25/38

30 very long-chain acyl-CoA synthetase isoform 1 70311.8

22749961

9

13 (13

0 0 0 0)

4472, 1-2 K.KDDVSIYYVSR.T 933.4 1 16/20

5370, 2-2 K.FSASQFWDDCRK.Y 674.8 1 12/22

5376, 2-2 K.FSASQFWDDCRK.Y 652.0 1 12/22

5681, 1-2 R.IQDTIEITGTFK.H 599.8 1 15/22

5964, 2-2 K.FSASQFWDDCR.K 1036.8 1 15/20

6369, 2-2 K.LFQHIADYLPSYAR.P 569.0 1 13/26

6370, 2-2 K.DALYFLDDTAK.M 991.4 1 16/20

6546, 1-2 K.DALYFLDDTAK.M 800.5 1 15/20

7308, 2-2 K.MYVPMTEDIYNAISAK.T 584.0 1 17/30

7425, 2-2 R.IWYGTGLTFVSGLK.A 785.8 1 17/26

7619, 1-2 R.SEVTFSTPALYIYTSGTTGLPK.A 521.7 1 18/42

Page 66: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7793, 1 R.IWYGTGLTFVSGLK.A 994.8 1 18/26

9924, 1 K.VLLVSPELQAAVEEILPSLK.K 850.8 1 18/38

31 NADPH--cytochrome P450 reductase 77047.7

12713903

3

11 (11

0 0 0 0)

3608, 2-2 K.SYENQKPPFDAK.N 850.6 1 18/22

3681, 1-2 K.SYENQKPPFDAK.N 754.6 1 17/22

5303, 2-2 K.IRYESGDHVAVYPANDSALVNQLGK.I

1107.3 1 29/96

5547, 2-2 R.SDEDYLYREELAQFHR.D 1643.7 1 30/60

5578, 2-2 K.EVGETLLYYGCR.R 777.9 1 15/22

5716, 1 R.YESGDHVAVYPANDSALVNQLGK.I 1023.2 1 32/88

5738, 1-2 K.EVGETLLYYGCR.R 664.3 1 14/22

6076, 2-2 R.DGALTQLNVAFSR.E 1453.5 1 18/24

6593, 1 R.NIIVFYGSQTGTAEEFANR.L 2081.5 1 27/36

7748, 2-2 R.TNVLYELAQYASEPSEQELLRK.M 735.4 1 27/84

8680, 1 R.TNVLYELAQYASEPSEQELLR.K 890.5 1 30/80

32 bifunctional ATP-dependent dihydroxyacetone kinase/FAD-AMP lyase (cyclizing) 58946.8 20149621

12 (12

0 0 0 0)

4260, 1-2 R.GLCGTVLIHK.V 741.3 1 14/18

6392, 2-2 R.LEQPDPGAVAAAAILR.A 676.4 1 19/30

6704, 1-2 K.VAGALAEAGVGLEEIAK.Q 2345.7 1 25/32

6918, 2-2 R.VCSTLLGLEEHLNALDR.A 595.7 1 16/32

7137, 1-2 R.VCSTLLGLEEHLNALDR.A 641.5 1 16/32

7150, 1-2 K.SPGADLLQVLTK.A 2271.3 1 19/22

7216, 1-2 K.LIDAETTAAAWPNVAAVSITGR.K 541.7 1 18/42

8411, 1-2 K.MGGSSGALYGLFLTAAAQPLK.A 1337.7 1 22/40

8446, 1-2 R.AEGIPVEMVVIGDDSAFTVLK.K 1017.2 1 22/40

8506, 1 K.MGGSSGALYGLFLTAAAQPLK.A 1007.3 1 20/40

8564, 1 R.AEGIPVEMVVIGDDSAFTVLK.K 822.8 1 21/40

9012, 1-2 R.TMLDSLWAAGQELQAWK.S 1336.8 1 20/32

33 dehydrogenase/reductase SDR family member 1 33908.8

20986290

1

23 (23

0 0 0 0)

4462, 1-2 K.SAFSSAETTELSGK.C 1960.5 1 21/26

5342, 1 R.VVAQEAQSLGGQCVPVVCDSSQESEVR.S

1104.4 1 35/104

5360, 1 R.VVAQEAQSLGGQCVPVVCDSSQESEVR.S

1120.0 1 23/52

6627, 2-2 K.WIIALYTSK.F 695.7 1 14/16

6651, 2-2 K.CVVALATDPNILSLSGK.V 2671.1 1 26/32

6677, 2-2 K.CVVALATDPNILSLSGK.V 2222.2 1 24/32

6864, 1-2 K.CVVALATDPNILSLSGK.V 2876.5 1 26/32

6917, 1 K.WIIALYTSK.F 779.5 1 15/16

6974, 1 K.CVVALATDPNILSLSGK.V 2543.4 1 25/32

7430, 2-2 R.HGVSCVSLWPGIVQTELLK.E 879.3 1 21/36

7659, 2-2 K.WIIALYTSKF.- 890.5 1 14/18

7690, 1-2 R.HGVSCVSLWPGIVQTELLK.E 1244.5 1 24/36

7700, 1-2 R.HGVSCVSLWPGIVQTELLK.E 1290.3 1 28/72

7784, 1 R.HGVSCVSLWPGIVQTELLK.E 1444.6 1 26/36

7796, 1 R.HGVSCVSLWPGIVQTELLK.E 1609.4 1 33/72

7802, 1 R.HGVSCVSLWPGIVQTELLK.E 1013.8 1 23/36

Page 67: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7815, 2-2 R.LDVLVNNAYAGVQTILNTR.N 2022.2 1 31/72

8080, 1-2 R.LDVLVNNAYAGVQTILNTR.N 2618.5 1 26/36

8204, 1 R.LDVLVNNAYAGVQTILNTR.N 1534.5 1 26/36

8278, 2-2 K.AFWETPASMWDDINNVGLR.G 620.9 1 18/36

8304, 1 R.LDVLVNNAYAGVQTILNTR.N 631.2 1 17/36

8604, 1 R.EQQGRLDVLVNNAYAGVQTILNTR.N

889.0 1 27/92

9683, 1 K.CVVALATDPNILSLSGK.V 541.7 1 14/32

34 UTP--glucose-1-phosphate uridylyltransferase isoform a 56939.9 48255966

23 (23

0 0 0 0)

3922, 2-2 K.IQRPPEDSIQPYEK.I 1044.8 1 25/52

3924, 2-2 K.IQRPPEDSIQPYEK.I 555.4 1 16/26

3998, 1-2 K.IQRPPEDSIQPYEK.I 1382.5 1 26/52

4228, 2-2 R.INKESLLPVAK.D 1441.1 1 17/20

4308, 1-2 R.INKESLLPVAK.D 1620.6 1 18/20

4544, 2-2 R.LVEIAQVPK.A 1116.0 1 15/16

4653, 1-2 R.LVEIAQVPK.A 1016.7 1 14/16

5634, 2-2 K.SFENSLGINVPR.S 1129.9 1 16/22

5782, 1-2 K.SFENSLGINVPR.S 867.1 1 14/22

5807, 2-2 K.GTVIIIANHGDRIDIPPGAVLENK.I 1189.3 1 30/92

5912, 1 K.SFENSLGINVPR.S 1317.2 1 17/22

5967, 1-2 K.GTVIIIANHGDRIDIPPGAVLENK.I 1156.2 1 34/92

7634, 2-2 R.NENTFLDLTVQQIEHLNK.T 1606.7 1 21/34

7779, 1-2 K.TYNTDVPLVLMNSFNTDEDTK.K 574.1 1 17/40

7870, 1-2 R.NENTFLDLTVQQIEHLNK.T 1707.7 1 22/34

7876, 1-2 R.NENTFLDLTVQQIEHLNK.T 827.8 1 27/68

7882, 1-2 R.NENTFLDLTVQQIEHLNK.T 1442.0 1 21/34

7923, 2-2 K.IFNTNNLWISLAAVK.R 1863.9 1 21/28

7994, 1 R.NENTFLDLTVQQIEHLNK.T 1038.3 1 18/34

8156, 2-2 K.TLDGGLNVIQLETAVGAAIK.S 1132.2 1 22/38

8421, 1-2 R.NENTFLDLTVQQIEHLNK.T 523.7 1 14/34

8483, 1 K.TLDGGLNVIQLETAVGAAIK.S 648.0 1 17/38

8523, 1 K.TLDGGLNVIQLETAVGAAIK.S 1920.6 1 33/76

35 cytochrome P450 2E1 precursor 56848.6 10834998

14 (14

0 0 0 0)

4007, 1-2 K.FKPEHFLNENGK.F 678.6 1 16/22

5228, 2-2 R.EAHFLLEALRK.T 1369.5 1 16/20

5285, 2-2 R.DLTDCLLVEM*EKEK.H 569.5 1 15/26

5364, 1-2 R.EAHFLLEALRK.T 1285.6 1 15/20

5795, 2-2 R.GIIFNNGPTWK.D 1228.0 1 16/20

6105, 2-2 K.DRQEMPYMDAVVHEIQR.F 724.9 1 28/64

6759, 2-2 R.DLTDCLLVEMEKEK.H 1703.3 1 21/26

6874, 2-2 R.FGPVFTLYVGSQR.M 1900.3 1 19/24

6959, 1-2 R.DLTDCLLVEMEKEK.H 506.2 1 13/26

7013, 2-2 K.DIDLSPIHIGFGCIPPR.Y 549.4 1 15/32

7095, 1-2 R.FGPVFTLYVGSQR.M 1657.2 1 19/24

7419, 2-2 K.GTVVVPTLDSVLYDNQEFPDPEK.F 1079.7 1 22/44

7683, 1-2 K.GTVVVPTLDSVLYDNQEFPDPEK.F 1498.5 1 24/44

7809, 1 K.GTVVVPTLDSVLYDNQEFPDPEK.F 519.0 1 17/44

36 formimidoyltransferase-cyclodeaminase 58926.2 11140815 13 (13

Page 68: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

0 0 0 0)

6039, 1-2 R.GVSVDECVLCAQAFGQR.L 584.4 1 15/32

6122, 1-2 K.TQAALVLDCLETRQE.- 1343.1 1 19/28

6135, 1-2 K.TQAALVLDCLETRQE.- 1394.3 1 19/28

6496, 2-2 R.LGLDSLCPFSPK.E 1139.5 1 16/22

6676, 1-2 R.LGLDSLCPFSPK.E 1466.1 1 18/22

7077, 1-2 R.MGALDVCPFIPVR.G 938.6 1 17/24

7977, 1 R.KFLIAFNINLLGTK.E 1769.8 1 21/26

7978, 1-2 R.TVYTFVGPPECVVEGALNAAR.V 525.0 1 19/40

8075, 1 R.TVYTFVGPPECVVEGALNAAR.V 503.8 1 19/40

8124, 1 K.LQQADWAPDFGPSSFVPSWGATATGAR.K

810.7 1 32/104

8660, 1-2 R.EAQELSLPVVGSQLVGLVPLK.A 1290.1 1 27/40

8746, 1 R.EAQELSLPVVGSQLVGLVPLK.A 1177.1 1 26/40

9958, 1 K.NLAQVSTNLLDFEVTALHTVYEETCR.E

702.3 1 17/50

37 tubulin alpha-1A chain 50135.3 17986283

14 (14

0 0 0 0)

5342, 2-2 R.QLFHPEQLITGKEDAANNYAR.G 1833.2 1 35/80

5502, 2-2 R.QLFHPEQLITGK.E 543.9 1 15/22

5586, 1 R.QLFHPEQLITGKEDAANNYAR.G 1080.0 1 30/80

5633, 2-2 K.VGINYQPPTVVPGGDLAK.V 559.0 1 20/34

5667, 1-2 R.QLFHPEQLITGK.E 696.4 1 17/22

5801, 1-2 K.VGINYQPPTVVPGGDLAK.V 541.2 1 19/34

6287, 2-2 R.AVCM*LSNTTAIAEAWAR.L 1320.0 1 20/32

6412, 2-2 R.IHFPLATYAPVISAEK.A 752.9 1 19/30

6604, 1-2 R.IHFPLATYAPVISAEK.A 766.9 1 19/30

6719, 1 R.IHFPLATYAPVISAEK.A 707.6 1 19/30

6840, 1 K.TIGGGDDSFNTFFSETGAGK.H 1512.8 1 23/38

7256, 1-2 R.AVFVDLEPTVIDEVR.T 630.8 1 18/28

7374, 1-2 R.AVCMLSNTTAIAEAWAR.L 1065.4 1 25/64

8252, 1 R.FDGALNVDLTEFQTNLVPYPR.I 891.9 1 20/40

38 serum albumin preproprotein 69366.4 4502027

14 (14

0 0 0 0)

4226, 1-2 R.FKDLGEENFK.A 1035.1 1 16/18

4562, 1 K.VHTECCHGDLLECADDRADLAK.Y 870.9 1 32/84

4827, 2-2 K.KVPQVSTPTLVEVSR.N 764.1 1 17/28

5133, 1 K.KVPQVSTPTLVEVSR.N 877.7 1 19/28

5895, 1 R.RPCFSALEVDETYVPK.E 520.2 1 16/30

6058, 2-2 K.SLHTLFGDKLCTVATLR.E 645.3 1 15/32

6116, 2-2 K.QNCELFEQLGEYK.F 1249.1 1 18/24

6250, 1 K.SLHTLFGDKLCTVATLR.E 1539.7 1 23/32

6268, 1 K.SLHTLFGDKLCTVATLR.E 1479.4 1 22/32

6366, 1 K.QNCELFEQLGEYK.F 1296.4 1 18/24

7160, 1 K.VFDEFKPLVEEPQNLIK.Q 1307.9 1 32/64

7690, 1 R.RHPYFYAPELLFFAK.R 1590.2 1 30/56

9092, 1 K.ALVLIAFAQYLQQCPFEDHVK.L 1358.7 1 20/40

9110, 1 K.ALVLIAFAQYLQQCPFEDHVK.L 1296.2 1 31/80

39 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581

15 (15

0 0 0 0)

5230, 2-2 K.DVEVTKEEFVLAAQK.F 2242.0 1 23/28

Page 69: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5498, 2-2 K.LAVATFAGIENK.F 1873.3 1 19/22

5638, 1-2 K.LAVATFAGIENK.F 1525.7 1 18/22

6180, 2-2 R.PVLLQVAESAYR.F 1555.0 1 18/22

6254, 2-2 K.ASGDSARPVLLQVAESAYR.F 1166.1 1 27/72

6339, 1-2 R.PVLLQVAESAYR.F 1289.9 1 18/22

6462, 1-2 K.ASGDSARPVLLQVAESAYR.F 571.8 1 19/36

7007, 2-2 R.IAPLEEGTLPFNLAEAQR.Q 760.4 1 22/34

7205, 1-2 R.IAPLEEGTLPFNLAEAQR.Q 791.7 1 23/34

7336, 1 R.IAPLEEGTLPFNLAEAQR.Q 842.1 1 22/34

7356, 1-2 R.DLGFFGIYK.G 610.1 1 13/16

7438, 1-2 R.DIPFSAIYFPCYAHVK.A 546.5 1 14/30

7654, 1-2 K.FGLYLPLFKPSVSTSK.A 526.6 1 18/30

8004, 2-2 R.FGLGSVAGAVGATAVYPIDLVK.T 958.2 1 20/42

8296, 1-2 R.FGLGSVAGAVGATAVYPIDLVK.T 1038.6 1 21/42

40 methyltransferase-like protein 7A precursor 28318.9 89145417

29 (29

0 0 0 0)

4424, 1-2 R.VTCIDPNPNFEK.F 781.1 1 16/22

4533, 2-2 R.FTVIYNEQM*ASK.K 1051.0 1 18/22

4628, 1 R.VTCIDPNPNFEK.F 715.3 1 16/22

4646, 1-2 R.FTVIYNEQM*ASK.K 859.8 1 17/22

4989, 2-2 R.FTVIYNEQMASK.K 843.0 1 17/22

5123, 1-2 R.FTVIYNEQMASK.K 977.7 1 18/22

5306, 1 R.FTVIYNEQMASK.K 878.0 1 17/22

5950, 1-2 K.RELFSNLQEFAGPSGK.L 833.6 1 18/30

6027, 1 K.RELFSNLQEFAGPSGK.L 581.4 2 14/30

6248, 2-2 K.LQHIQAPLSWELVR.P 3473.3 1 32/52

6308, 2-2 K.LSLLEVGCGTGANFK.F 1377.2 1 20/28

6356, 2-2 R.ELFSNLQEFAGPSGK.L 617.9 1 15/28

6410, 1-2 K.LSLLEVGCGTGANFK.F 1726.2 1 23/28

6416, 2-2 R.ELFSNLQEFAGPSGK.L 1380.7 1 19/28

6417, 1 K.LSLLEVGCGTGANFK.F 1122.2 1 18/28

6422, 2-2 K.LQHIQAPLSWELVRPHIYGYAVK.- 816.5 1 30/88

6443, 1-2 K.LQHIQAPLSWELVR.P 882.5 1 17/26

6490, 1 K.LQHIQAPLSWELVR.P 2972.8 1 31/52

6497, 1 K.LQHIQAPLSWELVR.P 1428.7 1 21/26

6584, 1-2 R.ELFSNLQEFAGPSGK.L 1713.2 1 21/28

6599, 1 K.LQHIQAPLSWELVRPHIYGYAVK.- 719.3 1 18/44

6614, 1 K.LQHIQAPLSWELVRPHIYGYAVK.- 787.3 1 32/88

6627, 1-2 K.LQHIQAPLSWELVRPHIYGYAVK.- 935.3 1 31/88

6668, 1 R.ELFSNLQEFAGPSGK.L 848.0 1 15/28

6672, 1 R.ELFSNLQEFAGPSGK.L 927.6 1 16/28

6735, 1 K.LSLLEVGCGTGANFK.F 1136.6 1 19/28

6771, 1 R.ELFSNLQEFAGPSGK.L 1603.1 1 20/28

7416, 1-2 -.M*ELTIFILR.L 775.1 2 13/16

10656, 1 K.LSLLEVGCGTGANFK.F 939.6 1 18/28

41 annexin A6 isoform 1 75872.8 71773329

12 (12

0 0 0 0)

3671, 1-2 K.GTVRPANDFNPDADAK.A 771.6 1 16/30

4931, 2-2 K.SLHQAIEGDTSGDFLK.A 1744.7 1 21/30

5225, 1 K.SLHQAIEGDTSGDFLK.A 2102.1 1 22/30

6288, 2-2 R.DAFVAIVQSVK.N 1934.2 1 18/20

Page 70: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

6365, 1-2 R.DLEADIIGDTSGHFQK.M 1377.6 1 22/30

6452, 1 R.DLEADIIGDTSGHFQK.M 1151.6 1 21/30

6467, 1-2 R.DAFVAIVQSVK.N 1818.9 1 17/20

6563, 1 K.CLIEILASR.T 718.7 1 14/16

6830, 1-2 R.LILGLMMPPAHYDAK.Q 524.3 1 16/28

7498, 2-2 K.WGTDEAQFIYILGNR.S 1994.4 1 22/28

7858, 1-2 K.GLGTDEDTIIDIITHR.S 1124.5 1 19/30

8326, 1 K.DLIADLKYELTGK.F 1328.1 1 18/24

42 programmed cell death 6-interacting protein isoform 1 96022.6 22027538

10 (10

0 0 0 0)

4382, 1-2 K.FIQQTYPSGGEEQAQYCR.A 522.1 1 20/34

4532, 1 K.FIQQTYPSGGEEQAQYCR.A 592.2 1 20/34

4559, 1-2 R.LLDEEEATDNDLR.A 1604.5 1 19/24

5243, 2-2 K.DAFDKGSLFGGSVK.L 806.8 1 17/26

5387, 1-2 K.LALASLGYEK.S 1273.2 1 15/18

5747, 1-2 K.MVPVSVQQSLAAYNQR.K 816.9 1 18/30

5861, 1-2 R.VPDLKDLDPIGK.A 756.6 2 16/22

6152, 2-2 K.LANQAADYFGDAFK.Q 1436.8 1 19/26

6746, 1 R.SVIEQGGIQTVDQLIK.E 885.6 1 16/30

7258, 2-2 K.FYNELTEILVR.F 1540.6 1 18/20

43 fatty aldehyde dehydrogenase isoform 1 57669.1 73466520

14 (13

1 0 0 0)

4510, 1-2 K.EFYGENIKESPDYER.I 818.7 1 16/28

4676, 1 K.EFYGENIKESPDYER.I 734.8 1 14/28

5434, 1 K.SPCYIDKDCDLDIVCR.R 1290.6 1 21/30

5444, 1-2 R.ILSLLEGQK.I 552.1 2 13/16

5679, 2-2 R.YIAPTVLTDVDPK.T 1050.3 1 18/24

5800, 1-2 R.YIAPTVLTDVDPK.T 1184.6 1 19/24

5858, 2-2 K.NVDEAINFINER.E 1872.8 1 19/22

5922, 1 R.YIAPTVLTDVDPK.T 737.4 1 16/24

6111, 1 K.NVDEAINFINER.E 1422.2 1 16/22

7293, 2-2 R.EKDILTAIAADLCK.S 1367.3 1 19/26

7520, 1-2 R.EKDILTAIAADLCK.S 1656.5 1 20/26

7623, 1 R.EKDILTAIAADLCK.S 1132.9 1 18/26

7756, 2-2 K.VM*QEEIFGPILPIVPVK.N 683.4 1 14/32

8256, 2-2 K.DILTAIAADLCK.S 1164.2 1 17/22

44 78 kDa glucose-regulated protein precursor 72332.5 16507237

12 (12

0 0 0 0)

4469, 2-2 R.TWNDPSVQQDIK.F 1013.5 1 17/22

4590, 1-2 R.TWNDPSVQQDIK.F 976.2 1 17/22

4862, 2-2 K.NQLTSNPENTVFDAK.R 846.8 1 18/28

5045, 1 K.LYGSAGPPPTGEEDTAEKDEL.- 904.1 1 20/40

5049, 2-2 K.SQIFSTASDNQPTVTIK.V 1416.7 1 20/32

5213, 1-2 K.SQIFSTASDNQPTVTIK.V 1243.9 1 22/32

5344, 1 K.SQIFSTASDNQPTVTIK.V 920.8 1 20/32

5538, 1 K.VTHAVVTVPAYFNDAQR.Q 622.6 1 18/32

5848, 2-2 K.SDIDEIVLVGGSTR.I 950.4 1 16/26

5949, 1-2 R.IINEPTAAAIAYGLDKR.E 1019.9 1 20/32

6014, 1 R.IINEPTAAAIAYGLDKR.E 1121.6 1 21/32

6656, 1-2 R.AKFEELNMDLFR.S 1231.1 1 17/22

45 amine oxidase [flavin-containing] B 58762.6 38202207 11 (11

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0 0 0 0)

4739, 2-2 K.YVDLGGSYVGPTQNR.I 948.1 1 21/28

4749, 2-2 K.LERPVIYIDQTR.E 1076.6 1 17/22

4853, 1-2 K.YVDLGGSYVGPTQNR.I 921.8 1 19/28

4868, 1-2 K.LERPVIYIDQTR.E 945.0 1 16/22

5650, 2-2 R.ENVLVETLNHEM*YEAK.Y 624.7 1 17/30

5688, 2-2 K.ELLDKLCWTESAK.Q 1136.2 1 19/24

5895, 2-2 K.VLGSLEALEPVHYEEK.N 878.5 1 16/30

5974, 1 K.LLHDSGLNVVVLEAR.D 950.3 1 21/28

6063, 1-2 K.VLGSLEALEPVHYEEK.N 946.3 1 17/30

6256, 2-2 K.YVISAIPPTLGMK.I 750.9 1 16/24

6581, 2-2 R.ENVLVETLNHEMYEAK.Y 1512.8 1 19/30

46 prelamin-A/C isoform 1 precursor 74139.0 27436946

8 (7 1 0

0 0)

4013, 1-2 K.AAYEAELGDAR.K 1537.0 1 17/20

4424, 1 K.ASASGSGAQVGGPISSGSSASSVTVTR.S

660.8 1 21/52

4738, 1 R.VAVEEVDEEGKFVR.L 723.2 1 15/26

5138, 1-2 R.TLEGELHDLRGQVAK.L 537.2 1 16/28

5164, 1-2 R.LADALQELR.A 1105.8 1 15/16

5822, 1-2 R.LKDLEALLNSK.E 1388.5 2 16/20

6226, 1 R.IRIDSLSAQLSQLQK.Q 994.3 1 17/28

7052, 1-2 R.MQQQLDEYQELLDIK.L 2038.2 1 22/28

47 endoplasmin precursor 92468.2 4507677

13 (12

0 0 0 1)

4002, 1-2 K.SREGSRTDDEVVQR.E 694.6 3 14/26

4937, 2-2 K.IADDKYNDTFWK.E 1326.3 1 17/22

5061, 1-2 K.IADDKYNDTFWK.E 1203.5 1 17/22

5112, 2-2 R.FQSSHHPTDITSLDQYVER.M 1845.1 1 32/72

5408, 1 R.FQSSHHPTDITSLDQYVER.M 742.9 1 19/36

5488, 1-2 K.GVVDSDDLPLNVSR.E 967.4 1 19/26

5612, 1 K.GVVDSDDLPLNVSR.E 1245.3 1 21/26

5762, 2-2 K.SILFVPTSAPR.G 1026.0 1 18/20

6466, 1 R.LISLTDENALSGNEELTVK.I 655.1 1 18/36

8094, 2-2 K.YSQFINFPIYVWSSK.T 586.9 1 14/28

8116, 2-2 K.YSQFINFPIYVWSSK.T 603.4 1 13/28

8369, 1-2 K.YSQFINFPIYVWSSK.T 828.5 1 15/28

9934, 1 R.IKEDEDDKTVLDLAVVLFETATLR.S 966.1 1 30/92

48 monoglyceride lipase isoform 1 34292.3 6005786

9 (9 0 0

0 0)

3852, 2-2 K.ALIFVSHGAGEHSGR.Y 535.5 1 14/28

5316, 2-2 R.NKTEVDIYNSDPLICR.A 952.2 1 20/30

6170, 1 R.MVVSDFHVFVR.D 871.3 2 14/20

6741, 2-2 K.GAYLLMELAK.S 1120.1 1 14/18

6766, 2-2 R.RTPQSIPYQDLPHLVNADGQYLFCR.Y

972.8 1 29/96

7089, 1 R.RTPQSIPYQDLPHLVNADGQYLFCR.Y

1278.0 1 34/96

7708, 2-2 K.LTVPFLLLQGSADR.L 552.8 1 15/26

7979, 1-2 K.LTVPFLLLQGSADR.L 842.7 1 18/26

8828, 1 K.VLNLVLPNLSLGPIDSSVLSR.N 1441.1 1 32/80

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49 aldehyde dehydrogenase family 8 member A1 isoform 3 48035.6

30150069

8

8 (8 0 0

0 0)

3791, 2-2 R.VPNSGKDEIEAAVK.A 901.4 1 17/26

5852, 2-2 K.YGLAATVWSSNVGR.V 1047.5 1 20/26

5999, 1-2 K.YGLAATVWSSNVGR.V 859.3 1 18/26

6657, 1-2 R.VGEALVSHPEVPLISFTGSQPTAER.I

846.9 1 30/96

6855, 1-2 K.DSYDFFTEIK.T 929.9 1 14/18

7209, 1-2 K.NPAIIFEDANLDECIPATVR.S 1534.1 1 26/38

8606, 1 K.VGIPSDPLVSIGALISK.A 649.6 1 20/32

10005, 1 R.VLNQVADLLEQSLEEFAQAESK.D 1323.3 1 22/42

50 D-beta-hydroxybutyrate dehydrogenase, mitochondrial precursor 38156.9 44680133

7 (7 0 0

0 0)

4414, 1-2 R.TVQLNVCSSEEVEK.V 968.6 1 17/26

6111, 1-2 K.QVAEVNLWGTVR.M 767.1 1 15/22

6167, 2-2 K.MWEELPEVVR.K 810.0 1 14/18

6297, 1 K.FGVEAFSDCLR.Y 763.0 1 15/20

7070, 1-2 R.TVQLNVCSSEEVEKVVEIVR.S 1231.8 1 21/38

8051, 1-2 K.VSVVEPGNFIAATSLYSPESIQAIAK.K

632.9 1 19/50

8156, 1-2 K.METYCSSGSTDTSPVIDAVTHALTATTPYTR.Y

892.5 1 32/120

51 acetyl-CoA carboxylase 2 precursor 276537.6

13414206

2

9 (9 0 0

0 0)

4824, 1-2 K.VLIANNGIAAVK.C 1165.4 1 17/22

4857, 2-2 K.EASFEYLQNEGER.L 940.2 1 15/24

4985, 1-2 K.EASFEYLQNEGER.L 671.5 1 14/24

5518, 2-2 R.VIQVENSHIILTGASALNK.V 1837.2 1 23/36

5825, 2-2 R.QILIASHLPSYELR.H 844.0 1 17/26

5835, 2-2 K.GTWQSGFFDHGSFK.E 715.5 1 15/26

5901, 2-2 K.FGAYIVDGLR.Q 858.1 1 14/18

6041, 1-2 K.FGAYIVDGLR.Q 802.5 1 14/18

6482, 1-2 R.KAESAEDFPILFR.Q 1262.7 1 18/24

52 talin-1 269764.4

22302941

0

8 (8 0 0

0 0)

4612, 1 R.LASEAKPAAVAAENEEIGSHIK.H 1961.9 1 33/84

6734, 1 K.AVAEQIPLLVQGVR.G 730.1 1 18/26

7294, 1 K.VGAIPANALDDGQWSQGLISAAR.M

818.0 1 22/44

7359, 1-2 K.EADESLNFEEQILEAAK.S 1113.7 1 19/32

7871, 1-2 K.AGFLDLKDFLPK.E 1073.3 1 14/22

7997, 1-2 K.TLAESALQLLYTAK.E 1543.0 1 19/26

9336, 1 R.GVGAAATAVTQALNELLQHVK.A 788.1 1 19/40

9378, 1 R.GVGAAATAVTQALNELLQHVK.A 576.8 1 16/40

53 protein diaphanous homolog 1 isoform 1 141346.4

11939575

8

9 (9 0 0

0 0)

3514, 2-2 K.LQDLQGEKDALHSEK.Q 1646.4 1 20/28

6120, 2-2 R.VQLNVFDEQGEEDSYDLK.G 515.8 1 17/34

6228, 2-2 K.LVAEDLSQDCFWTK.V 823.9 1 17/26

6495, 1 K.LVAEDLSQDCFWTK.V 748.0 1 15/26

7022, 1 K.AGCAVTSLLASELTK.D 1038.8 1 20/28

Page 73: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7035, 1-2 K.QQIATEKQDLEAEVSQLTGEVAK.L 902.4 1 27/88

7599, 1-2 K.TAQNLSIFLGSFR.M 651.3 1 16/24

7733, 1 K.TAQNLSIFLGSFR.M 796.1 1 15/24

9212, 1 K.VGCLQLINALITPAEELDFR.V 855.4 1 20/38

54 C-1-tetrahydrofolate synthase, cytoplasmic 101530.5

22213663

9

8 (8 0 0

0 0)

5290, 2-2 K.TDTESELDLISR.L 1685.6 1 17/22

5476, 2-2 K.AYIQENLELVEK.G 1216.4 1 17/22

5686, 2-2 K.YVVVTGITPTPLGEGK.S 967.2 1 20/30

5866, 1 K.GCLELIKETGVPIAGR.H 1082.6 1 18/30

6243, 2-2 R.LDIDPETITWQR.V 964.8 1 17/22

6431, 1-2 R.LDIDPETITWQR.V 1079.5 1 18/22

7245, 1-2 R.AAQAPSSFQLLYDLK.L 862.8 1 20/28

7264, 1 R.EIGLLSEEVELYGETK.A 2170.2 1 23/30

55 cullin-associated NEDD8-dissociated protein 1 136374.7 21361794

12 (12

0 0 0 0)

5726, 2-2 K.ALTLIAGSPLK.I 1564.8 1 16/20

5819, 1-2 K.VIRPLDQPSSFDATPYIK.D 757.1 1 18/34

5871, 1-2 K.ALTLIAGSPLK.I 1178.4 1 15/20

6008, 2-2 R.AVAALLTIPEAEK.S 956.7 1 17/24

7535, 2-2 K.ADVFHAYLSLLK.Q 902.5 1 14/22

7685, 2-2 K.LGTLSALDILIK.N 842.7 1 18/22

7785, 1-2 K.ADVFHAYLSLLK.Q 1341.6 1 17/22

7820, 1-2 K.LTLIDPETLLPR.L 794.8 1 15/22

7919, 1 K.ADVFHAYLSLLK.Q 1527.8 1 17/22

8068, 1 K.LGTLSALDILIK.N 1002.0 1 18/22

8981, 2-2 K.ISGSILNELIGLVR.S 980.0 1 17/26

9245, 1 K.ISGSILNELIGLVR.S 1371.6 1 19/26

56 coatomer subunit alpha isoform 2 138344.7

14853685

3

8 (8 0 0

0 0)

3969, 2-2 K.YAVTTGDHGIIR.T 1864.6 1 19/22

5555, 2-2 R.SSGLTAVWVAR.N 1481.7 1 17/20

5828, 2-2 R.ASNLENSTYDLYTIPK.D 1664.9 1 22/30

6388, 1-2 K.SGAWDESGVFIYTTSNHIK.Y 677.3 1 18/36

6464, 1-2 R.TLDLPIYVTR.V 821.8 1 15/18

6608, 2-2 R.GITGVDLFGTTDAVVK.H 1455.7 1 21/30

6813, 1-2 R.GITGVDLFGTTDAVVK.H 1366.2 1 21/30

8907, 1 K.LSFLYLITGNLEK.L 1826.3 1 19/24

57 cytochrome P450 2A7 isoform 2 precursor 50666.4 15147328

9 (9 0 0

0 0)

5600, 2-2 K.KSDAFVPFSIGK.R 842.7 1 17/22

5727, 1-2 K.KSDAFVPFSIGK.R 1140.0 1 17/22

5896, 2-2 R.NYTMSFLPR.- 869.4 1 13/16

6225, 2-2 R.FGDVIPMSLAR.R 680.4 1 17/20

6442, 1-2 K.SDAFVPFSIGK.R 1022.6 1 16/20

6668, 2-2 K.MPYMEAVIHEIQR.F 1689.4 1 19/24

7350, 2-2 R.YGFLLLMK.H 849.5 1 12/14

7623, 2-2 R.TVSNVISSIVFGDR.F 1636.4 1 21/26

7996, 1 R.TVSNVISSIVFGDR.F 1507.4 1 21/26

58 perilipin-4 134430.4

12293719

5

8 (6 2 0

0 0)

Page 74: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

3714, 1-2 R.LDQGSGASAEDAAVQEER.D 1792.2 1 22/34

4644, 2-2 K.NTVCSGVTGAVNLAK.E 1275.3 1 17/28

4763, 1-2 K.NTVCSGVTGAVNLAK.E 1819.1 1 22/28

4763, 1-2 K.DTVTTGVM*GAVNLAK.G 659.0 2 16/28

4797, 1 R.ARPAADPTGAPAAEAAQPQAQVAAHPEQTAPWTEK.E

884.8 1 39/136

4899, 1 K.DAVTTGVTGAVNVAK.G 2280.7 1 23/28

4899, 1 K.DAVSTGLTGAVNVAK.G 1490.3 2 20/28

4954, 2-2 R.AKDAVSSGVASVVDVAK.G 964.2 1 19/32

59 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial 72691.1

15641600

3

9 (9 0 0

0 0)

4398, 2-2 R.TGHSLLHTLYGR.S 1246.3 1 17/22

4479, 1-2 R.TGHSLLHTLYGR.S 1531.4 1 18/22

4604, 2-2 K.VTLEYRPVIDK.T 1063.9 1 14/20

4725, 1-2 K.VTLEYRPVIDK.T 1086.0 1 15/20

5585, 1 R.HVNGQDQIVPGLYACGEAACASVHGANR.L

2275.9 1 38/108

5597, 1-2 R.HVNGQDQIVPGLYACGEAACASVHGANR.L

694.5 1 30/108

5898, 2-2 R.AAFGLSEAGFNTACVTK.L 1491.7 1 21/32

7803, 1 R.AGLPCQDLEFVQFHPTGIYGAGCLITEGCR.G

674.3 1 36/116

8926, 1-2 R.LGANSLLDLVVFGR.A 1466.6 1 19/26

60 cytochrome P450 2C9 precursor 55627.6 13699818

14 (14

0 0 0 0)

4004, 2-2 R.SHM*PYTDAVVHEVQR.Y 750.3 1 24/56

4449, 2-2 R.SHMPYTDAVVHEVQR.Y 728.7 1 18/28

6818, 2-2 R.YIDLLPTSLPHAVTCDIK.F 620.1 1 21/34

7017, 1-2 R.YIDLLPTSLPHAVTCDIK.F 704.8 1 22/34

7148, 1 R.YIDLLPTSLPHAVTCDIK.F 552.6 1 19/34

7545, 1-2 K.GTTILISLTSVLHDNK.E 809.9 1 15/30

7635, 1 K.GTTILISLTSVLHDNK.E 884.6 1 16/30

8162, 2-2 R.GKLPPGPTPLPVIGNILQIGIK.D 1146.0 1 27/84

8518, 1 R.GKLPPGPTPLPVIGNILQIGIK.D 1909.5 1 34/84

8810, 2-2 K.LPPGPTPLPVIGNILQIGIK.D 1428.1 1 30/76

8852, 2-2 K.LPPGPTPLPVIGNILQIGIK.D 838.0 1 23/38

9111, 1-2 K.LPPGPTPLPVIGNILQIGIK.D 927.9 1 24/38

9122, 1 K.LPPGPTPLPVIGNILQIGIK.D 1275.7 1 29/76

9146, 1 K.LPPGPTPLPVIGNILQIGIK.D 835.5 1 24/38

61 perilipin-3 isoform 1 47074.7

25595828

2

11 (11

0 0 0 0)

3261, 2-2 K.QLQGPEKEPPKPEQVESR.A 1150.7 1 29/68

3336, 1-2 K.QLQGPEKEPPKPEQVESR.A 1509.5 1 33/68

4798, 2-2 K.SVVTGGVQSVMGSR.L 2251.6 1 21/26

5104, 2-2 R.TLTAAAVSGAQPILSK.L 1704.1 1 21/30

5258, 1-2 R.TLTAAAVSGAQPILSK.L 1576.5 1 21/30

5404, 1 R.TLTAAAVSGAQPILSK.L 1839.2 1 22/30

5820, 2-2 K.DTVATQLSEAVDATR.G 2371.2 1 23/28

5992, 1-2 K.DTVATQLSEAVDATR.G 2439.5 1 23/28

5993, 1-2 K.SEEWADNHLPLTDAELAR.I 504.8 1 16/34

6040, 1 K.SEEWADNHLPLTDAELAR.I 1017.8 1 27/68

Page 75: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7995, 1 R.RQVEDLQATFSSIHSFQDLSSSILAQSR.E

1169.4 1 33/108

62 NADH-cytochrome b5 reductase 3 isoform 2 31628.5

19379482

6

11 (11

0 0 0 0)

5336, 1-2 R.STPAITLESPDIK.Y 1004.2 1 16/24

5459, 1 R.STPAITLESPDIK.Y 1125.6 1 17/24

5874, 2-2 R.STPAITLESPDIKYPLR.L 1239.6 1 21/32

6078, 1-2 R.STPAITLESPDIKYPLR.L 809.9 1 16/32

6116, 1 R.STPAITLESPDIKYPLR.L 837.7 1 18/32

6266, 2-2 K.DILLRPELEELR.N 665.0 1 14/22

6434, 1-2 K.DILLRPELEELR.N 913.0 1 16/22

6896, 2-2 K.MSQYLESMQIGDTIEFR.G 1335.1 1 19/32

6909, 2-2 R.IDGNLVVRPYTPISSDDDKGFVDLVIK.V

986.3 1 34/104

7020, 1-2 R.APEAWDYGQGFVNEEMIR.D 1452.4 1 22/34

7144, 1-2 R.IDGNLVVRPYTPISSDDDKGFVDLVIK.V

689.7 1 30/104

63 bile salt sulfotransferase 33779.6 29540545

12 (12

0 0 0 0)

6233, 2-2 K.NHFTVAQAEDFDKLFQEK.M 939.3 1 29/68

6244, 2-2 K.NHFTVAQAEDFDKLFQEK.M 1991.2 1 21/34

6468, 1 K.NHFTVAQAEDFDKLFQEK.M 872.0 1 30/68

6472, 1 K.NHFTVAQAEDFDKLFQEK.M 3003.9 1 26/34

6586, 2-2 R.SPWVESEIGYTALSETESPR.L 1853.8 1 24/38

6778, 1-2 R.SPWVESEIGYTALSETESPR.L 1263.7 1 23/38

6782, 2-2 R.LFSSHLPIQLFPK.S 549.5 1 14/24

6791, 2-2 R.LFSSHLPIQLFPK.S 718.0 1 24/48

6899, 1 R.SPWVESEIGYTALSETESPR.L 2420.3 1 29/38

7641, 1-2 K.NFLLLSYEELK.Q 857.7 1 15/20

8210, 1 R.DVLVSGYFFWK.N 1098.7 1 15/20

9260, 1 K.SGTNWLAEILCLMHSK.G 1017.7 1 18/30

64 protein disulfide-isomerase precursor 57115.9 20070125

7 (7 0 0

0 0)

3976, 2-2 K.LGETYKDHENIVIAK.M 1815.4 1 30/56

5163, 1-2 K.VDATEESDLAQQYGVR.G 2109.8 1 21/30

5549, 2-2 K.YQLDKDGVVLFK.K 1082.2 1 16/22

5792, 1 K.YQLDKDGVVLFK.K 1282.2 1 17/22

6064, 2-2 K.THILLFLPK.S 1121.7 1 14/16

6616, 2-2 R.EADDIVNWLK.K 815.8 1 15/18

9945, 1 R.TGPAATTLPDGAAAESLVESSEVAVIGFFK.D

615.1 1 19/58

65 protein transport protein Sec23A 86160.3 38202214

7 (7 0 0

0 0)

4842, 1-2 K.ISGAIGPCVSLNSK.G 549.4 1 16/26

4974, 1-2 R.GAIQFVTQYQHSSGQR.R 1308.7 1 20/30

5991, 1 K.ERPDLPPIQYEPVLCSR.T 512.7 1 22/64

6983, 1-2 R.SSFLQVFNNSPDESSYYR.H 2192.4 1 25/34

7109, 1 R.SSFLQVFNNSPDESSYYR.H 544.5 1 15/34

8079, 2-2 R.MVVPVAALFTPLK.E 817.2 1 17/24

8745, 1 K.IDMNLTDLLGELQR.D 641.9 1 14/26

66 liver carboxylesterase 1 isoform c precursor 62392.5 68508957 12 (12

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0 0 0 0)

5115, 2-2 R.QKTEEELLETTLK.M 822.9 1 14/24

5259, 1-2 R.QKTEEELLETTLK.M 662.1 1 13/24

5271, 2-2 R.GNWGHLDQVAALR.W 1818.2 1 19/24

5529, 1 R.GNWGHLDQVAALR.W 1746.0 1 19/24

6400, 1-2 K.AGQLLSELFTNRK.E 841.9 1 16/24

7134, 2-2 K.LSEDCLYLNIYTPADLTK.K 1229.5 1 19/34

7368, 1-2 K.LSEDCLYLNIYTPADLTK.K 1027.3 1 18/34

7606, 1-2 R.LGIWGFFSTGDEHSR.G 975.8 1 16/28

7726, 1 R.LGIWGFFSTGDEHSR.G 972.4 1 18/28

9192, 2-2 R.ESQPLLGTVIDGMLLLK.T 884.5 1 21/32

9323, 1 R.ESQPLLGTVIDGMLLLK.T 549.9 1 17/32

9392, 1 R.ESQPLLGTVIDGMLLLK.T 591.8 1 16/32

67 ribosome-binding protein 1 108654.8

11061122

0

6 (6 0 0

0 0)

4046, 2-2 R.DALNQATSQVESK.Q 1156.8 1 17/24

4558, 1 K.LREAEETQSTLQAECDQYR.S 500.5 1 16/36

4866, 2-2 R.TLQEQLENGPNTQLAR.L 1124.0 1 18/30

5043, 1 K.SHVEDGDIAGAPASSPEAPPAEQDPVQLK.T

776.2 1 35/112

5376, 1 R.QLLLESQSQLDAAK.S 756.3 1 16/26

7551, 2-2 K.VEPAVSSVVNSIQVLTSK.A 544.3 1 18/34

68 apolipoprotein A-I preproprotein 30777.6 4557321

6 (6 0 0

0 0)

5488, 2-2 K.DSGRDYVSQFEGSALGK.Q 836.9 1 17/32

6087, 2-2 R.VKDLATVYVDVLK.D 1492.6 1 19/24

6100, 1 R.DYVSQFEGSALGK.Q 1614.7 1 19/24

6585, 2-2 K.LLDNWDSVTSTFSK.L 1095.1 1 18/26

6911, 2-2 K.DLATVYVDVLK.D 1218.5 1 17/20

7762, 2-2 K.VSFLSALEEYTK.K 1437.9 1 19/22

69 calnexin precursor 67567.8 10716563

10 (10

0 0 0 0)

5121, 1-2 K.TPELNLDQFHDK.T 1320.6 1 17/22

5621, 2-2 K.CGEDYKLHFIFR.H 1480.7 1 25/44

5792, 1-2 R.IVDDWANDGWGLKK.A 1508.7 1 19/26

6164, 2-2 R.GTLSGWILSK.A 1169.3 1 15/18

6332, 1-2 R.GTLSGWILSK.A 1191.6 1 15/18

6347, 1-2 R.GTLSGWILSK.A 1275.3 1 16/18

7382, 2-2 R.KIPNPDFFEDLEPFR.M 964.8 1 17/28

7396, 2-2 R.KIPNPDFFEDLEPFR.M 1677.7 1 29/56

7656, 1-2 R.KIPNPDFFEDLEPFR.M 776.8 1 16/28

7991, 2-2 K.IPNPDFFEDLEPFR.M 1032.2 1 17/26

70 vimentin 53651.3 62414289

9 (9 0 0

0 0)

4098, 1-2 K.FADLSEAANR.N 1037.1 1 16/18

4850, 2-2 R.LQDEIQNMKEEMAR.H 1026.2 2 16/26

5889, 2-2 K.ILLAELEQLKGQGK.S 609.2 1 17/26

6232, 2-2 R.KVESLQEEIAFLK.K 1490.6 1 17/24

6767, 2-2 K.ILLAELEQLK.G 1309.0 1 16/18

6976, 1-2 K.ILLAELEQLK.G 1160.9 1 16/18

7079, 1 K.ILLAELEQLK.G 1152.8 1 15/18

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8056, 1-2 R.ISLPLPNFSSLNLR.E 916.0 1 20/26

8163, 1 R.ISLPLPNFSSLNLR.E 900.2 1 19/26

71 D-3-phosphoglycerate dehydrogenase 56650.2 23308577

8 (8 0 0

0 0)

4476, 1-2 R.KILQDGGLQVVEK.Q 1238.9 1 17/24

4733, 2-2 K.ILQDGGLQVVEK.Q 545.4 1 15/22

5308, 1-2 K.VLISDSLDPCCR.K 622.7 1 16/22

6201, 1-2 K.TLGILGLGR.I 860.6 1 14/16

7476, 1-2 R.CGEEIAVQFVDMVK.G 1145.0 1 18/26

7718, 2-2 K.NAGNCLSPAVIVGLLK.E 1009.9 1 21/30

7972, 1-2 K.NAGNCLSPAVIVGLLK.E 944.6 1 20/30

7994, 1-2 K.NAGNCLSPAVIVGLLK.E 567.3 1 16/30

72 protein ERGIC-53 precursor 57548.6 5031873

9 (9 0 0

0 0)

4671, 2-2 R.YVSSLTEEISK.R 613.0 1 15/20

5211, 2-2 K.NNPAIVIIGNNGQIHYDHQNDGASQALASCQR.D

1819.0 1 41/124

5370, 1-2 K.NNPAIVIIGNNGQIHYDHQNDGASQALASCQR.D

1102.5 1 36/124

5403, 2-2 K.YQEEFEHFQQELDK.K 1641.2 1 20/26

5486, 1 K.NNPAIVIIGNNGQIHYDHQNDGASQALASCQR.D

1355.5 1 39/124

5548, 1-2 K.YQEEFEHFQQELDK.K 1863.4 1 20/26

6032, 2-2 K.GPHLVQSDGTVPFWAHAGNAIPSSDQIR.V

1061.0 1 35/108

6285, 1 K.GPHLVQSDGTVPFWAHAGNAIPSSDQIR.V

749.1 1 33/108

6292, 1-2 K.GHPDLQGQPAEEIFESVGDR.E 847.3 1 26/76

73 amine oxidase [flavin-containing] A 59681.3 4557735

9 (9 0 0

0 0)

4359, 2-2 K.IHFRPELPAER.N 594.1 1 14/20

4810, 2-2 R.NEHVDYVDVGGAYVGPTQNR.I 2466.3 1 26/38

4941, 1-2 R.NEHVDYVDVGGAYVGPTQNR.I 533.1 1 26/76

4949, 1-2 R.NEHVDYVDVGGAYVGPTQNR.I 2263.5 1 25/38

4962, 1-2 R.IFFAGTETATK.W 916.8 1 17/20

5117, 1 R.NEHVDYVDVGGAYVGPTQNR.I 2425.3 1 26/38

5759, 1 K.LNHPVTHVDQSSDNIIIETLNHEHYECK.Y

1439.5 1 36/108

6634, 2-2 K.DVPAVEITHTFWER.N 660.2 1 16/26

6698, 2-2 K.DVPAVEITHTFWER.N 687.4 1 18/26

74 tubulin beta-2C chain 49830.7 5174735

8 (8 0 0

0 0)

3852, 1-2 -.M*REIVHLQAGQCGNQIGAK.F 791.6 1 22/72

4022, 2-2 R.EIVHLQAGQCGNQIGAK.F 2424.0 1 25/32

4023, 2-2 R.EIVHLQAGQCGNQIGAK.F 2023.2 1 31/64

4104, 1-2 R.EIVHLQAGQCGNQIGAK.F 1975.4 1 30/64

4134, 1-2 R.EIVHLQAGQCGNQIGAK.F 1002.4 1 22/32

4305, 1-2 R.INVYYNEATGGK.Y 855.3 1 16/22

6567, 1 K.FWEVISDEHGIDPTGTYHGDSDLQLER.I

1045.1 1 35/104

Page 78: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7094, 1-2 K.EAESCDCLQGFQLTHSLGGGTGSGMGTLLISK.I

1051.2 1 31/124

75 microsomal glutathione S-transferase 1 17598.6 9945306

11 (11

0 0 0 0)

4318, 2-2 R.KVFANPEDCVAFGKGENAK.K 1538.0 1 32/72

4329, 2-2 R.KVFANPEDCVAFGKGENAK.K 1159.4 1 21/36

4431, 1-2 R.KVFANPEDCVAFGKGENAK.K 2123.5 1 35/72

4700, 2-2 R.KVFANPEDCVAFGK.G 809.5 1 17/26

4820, 1-2 R.KVFANPEDCVAFGK.G 785.1 1 17/26

5193, 2-2 K.VFANPEDCVAFGK.G 884.5 1 18/24

5342, 1-2 K.VFANPEDCVAFGK.G 629.8 1 16/24

5487, 1 K.VFANPEDCVAFGK.G 1064.1 1 20/24

5690, 2-2 R.IYHTIAYLTPLPQPNR.A 2024.7 1 23/30

5823, 1-2 R.IYHTIAYLTPLPQPNR.A 2268.7 1 24/30

6705, 1-2 K.MMLMSTATAFYR.L 1208.5 1 17/22

76 tubulin beta chain 49670.5 29788785

7 (7 0 0

0 0)

4214, 2-2 R.ISVYYNEATGGK.Y 1126.2 1 17/22

4290, 1-2 R.ISVYYNEATGGK.Y 1279.9 1 19/22

6299, 2-2 K.FWEVISDEHGIDPTGTYHGDSDLQLDR.I

1248.1 1 33/104

7287, 1-2 R.ALTVPELTQQVFDAK.N 640.9 1 20/28

7709, 2-2 K.M*AVTFIGNSTAIQELFK.R 1144.4 1 21/32

8064, 2-2 K.MAVTFIGNSTAIQELFK.R 1384.2 1 22/32

8349, 1-2 K.MAVTFIGNSTAIQELFK.R 1308.9 1 21/32

77 complement C3 precursor 187146.3

11529867

8

5 (5 0 0

0 0)

4898, 1 K.LSINTHPSQKPLSITVR.T 904.6 1 26/64

5328, 1 K.VYAYYNLEESCTR.F 929.0 1 19/24

5910, 1 R.VPVAVQGEDTVQSLTQGDGVAK.L 609.7 1 23/42

6348, 1 R.LVAYYTLIGASGQR.E 1248.9 1 20/26

7696, 1 K.EYVLPSFEVIVEPTEK.F 973.1 1 21/30

78 sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating 41900.0 8393516

10 (10

0 0 0 0)

3818, 2-2 K.GVNTVFHCASPPPSSNNK.E 518.3 1 18/34

3894, 1-2 K.GVNTVFHCASPPPSSNNK.E 688.8 1 19/34

5296, 1 R.VALAGTFHYYSCER.A 1293.5 1 19/26

5307, 1 R.VALAGTFHYYSCER.A 1374.2 1 18/26

6416, 1 R.FFLGDLCSR.Q 767.6 1 14/16

6983, 2-2 R.GYAVNVFDIQQGFDNPQVR.F 1697.4 1 22/36

7223, 1-2 R.GYAVNVFDIQQGFDNPQVR.F 2190.7 1 24/36

7340, 2-2 R.DPQLVPILIEAAR.N 810.6 1 20/24

7348, 1 R.GYAVNVFDIQQGFDNPQVR.F 1621.2 1 31/72

7575, 1-2 R.DPQLVPILIEAAR.N 745.5 1 19/24

79 dimethylaniline monooxygenase [N-oxide-forming] 5 isoform 1 60220.2

22131667

2

8 (8 0 0

0 0)

4160, 2-2 R.ALSQHPTLNDDLPNR.I 564.8 1 17/28

5711, 2-2 K.EFTETAAIFEDGSR.E 861.9 1 15/26

5760, 2-2 R.IAVIGGGVSGLSSIK.C 1159.0 1 21/28

6009, 1 R.IAVIGGGVSGLSSIK.C 1299.6 1 22/28

6317, 2-2 K.LALHLLLGPCTPIHYR.V 556.7 1 16/30

Page 79: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

6518, 1-2 K.LALHLLLGPCTPIHYR.V 2087.4 1 31/60

6525, 1 K.LALHLLLGPCTPIHYR.V 1706.5 1 29/60

6640, 2-2 R.VGDYGYPADVLFSSR.L 558.9 1 18/28

80 glycogen debranching enzyme isoform 1 174762.1

11673484

7

5 (5 0 0

0 0)

5500, 1 R.LEQGYELQFR.L 813.6 1 14/18

6316, 1 K.YTWNDVGQLVEK.L 682.0 1 15/22

6496, 1 K.WIQEHPECAYNLVNSPHLKPAWVLDR.A

1124.8 1 35/100

6993, 1 R.HGLIPNLLGEGIYAR.Y 642.0 1 19/28

7797, 1 K.LWEFFQVDVNK.A 1207.8 1 16/20

81 elongation factor 2 95337.6 4503483

10 (10

0 0 0 0)

4972, 2-2 R.ETVSEESNVLCLSK.S 1803.7 1 19/26

5104, 1-2 R.ETVSEESNVLCLSK.S 1516.5 1 19/26

7574, 1 R.KIWCFGPDGTGPNILTDITK.G 684.2 1 24/76

7583, 1 R.KIWCFGPDGTGPNILTDITK.G 712.8 1 18/38

7937, 2-2 R.TFCQLILDPIFK.V 701.7 1 14/22

8195, 1-2 R.TFCQLILDPIFK.V 1111.7 1 17/22

8315, 1 R.TFCQLILDPIFK.V 1340.6 1 18/22

8433, 1-2 R.ALLELQLEPEELYQTFQR.I 729.0 1 19/34

8553, 1 R.ALLELQLEPEELYQTFQR.I 519.9 1 16/34

8751, 1 R.YVEPIEDVPCGNIVGLVGVDQFLVK.T

1252.3 1 32/96

82 NAD(P) transhydrogenase, mitochondrial precursor 113894.8

12293915

5

5 (5 0 0

0 0)

4142, 2-2 K.AVVLAANHFGR.F 1480.0 1 17/20

4882, 1-2 K.QGFNVVVESGAGEASK.F 1049.1 1 20/30

5860, 2-2 R.EANSIIITPGYGLCAAK.A 975.0 1 20/32

6222, 1-2 K.ILIVGGGVAGLASAGAAK.S 1075.3 1 20/34

7694, 2-2 R.VTIAQGYDALSSMANIAGYK.A 1271.5 1 19/38

83 serum paraoxonase/arylesterase 1 precursor 39731.0 19923106

8 (8 0 0

0 0)

6519, 2-2 K.IFFYDSENPPASEVLR.I 1070.1 1 22/30

6628, 2-2 R.VVAEGFDFANGINISPDGK.Y 1598.7 1 21/36

6708, 1-2 K.YVYIAELLAHK.I 1382.0 1 17/20

6731, 1-2 K.IFFYDSENPPASEVLR.I 675.8 1 18/30

6796, 1-2 R.VVAEGFDFANGINISPDGK.Y 773.1 1 18/36

8045, 2-2 K.ILLMDLNEEDPTVLELGITGSK.F 519.7 1 15/42

8544, 2-2 K.GIETGSEDLEILPNGLAFISSGLK.Y 1429.7 1 32/92

8901, 1 K.GIETGSEDLEILPNGLAFISSGLK.Y 1606.9 1 31/92

84 cytochrome P450 2C8 isoform b 47785.8

31189330

9

8 (7 1 0

0 0)

4484, 2-2 R.VQEEAHCLVEELRK.T 1086.0 1 16/26

4492, 1-2 K.SVDDLKNLNTTAVTK.G 1479.9 1 21/28

4706, 2-2 K.EALIDNGEEFSGR.G 690.8 1 16/24

4830, 1-2 K.EALIDNGEEFSGR.G 802.2 1 17/24

6396, 2-2 K.DQNFLTLMK.R 639.1 2 13/16

6682, 2-2 K.EALIDNGEEFSGR.G 1146.5 2 18/24

6874, 1-2 K.EALIDNGEEFSGR.G 1020.7 1 18/24

Page 80: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

8810, 1 R.ILNSPWIQVCNNFPLLIDCFPGTHNK.V

603.2 1 25/100

85 coatomer subunit beta 107141.5 7705369

7 (6 1 0

0 0)

5934, 2-2 K.YEAAGTLVTLSSAPTAIK.A 1329.8 1 22/34

6093, 1-2 K.YEAAGTLVTLSSAPTAIK.A 610.8 1 18/34

6202, 1 R.SIFGEDALANVSIEKPIHQGPDAAVTGHIR.I

873.7 1 32/116

6436, 1-2 R.NAFMMLIHADQDR.A 520.6 1 14/24

6797, 2-2 K.TLQLALDLVSSR.N 1722.6 1 17/22

7088, 1-2 K.AAAQCYIDLIIK.E 783.2 1 13/22

7236, 1 K.AAAQCYIDLIIK.E 1134.0 4 16/22

86 voltage-dependent anion-selective channel protein 1 30772.3 4507879

9 (9 0 0

0 0)

4667, 2-2 K.LTFDSSFSPNTGKK.N 770.0 1 16/26

4788, 1-2 K.LTFDSSFSPNTGKK.N 622.3 1 15/26

5751, 2-2 K.VNNSSLIGLGYTQTLKPGIK.L 652.2 1 18/38

5962, 2-2 R.WTEYGLTFTEK.W 944.1 1 15/20

5984, 1 K.VNNSSLIGLGYTQTLKPGIK.L 534.0 1 17/38

6119, 1-2 R.WTEYGLTFTEK.W 739.8 1 13/20

6648, 2-2 K.LTLSALLDGK.N 869.5 1 15/18

6684, 1 K.WNTDNTLGTEITVEDQLAR.G 1170.4 1 21/36

6851, 1-2 K.LTLSALLDGK.N 938.2 1 14/18

87 villin-1 92694.4

19439423

7

7 (7 0 0

0 0)

4710, 1-2 K.LYHVSDSEGNLVVR.E 1249.1 1 20/26

5921, 1-2 K.LIIQWNGPESTR.M 762.3 1 16/22

6298, 1-2 R.DWSQITAEVTSPK.V 1318.8 1 18/24

6400, 1 R.DWSQITAEVTSPK.V 959.1 1 16/24

6639, 1-2 R.EVATRPLTQDLLSHEDCYILDQGGLK.I

755.4 1 26/100

7048, 1-2 R.ANFLNSNDVFVLK.T 1175.2 1 18/24

7175, 1 R.ANFLNSNDVFVLK.T 854.8 1 16/24

88 cytochrome P450 2C19 precursor 55944.8 4503219

10 (9 1

0 0 0)

4823, 2-2 R.MVVLHGYEVVK.E 1010.2 1 15/20

4947, 1-2 R.MVVLHGYEVVK.E 936.3 1 15/20

5348, 2-2 R.RFSLMTLR.N 1118.0 1 13/14

5487, 1-2 R.RFSLMTLR.N 1148.9 1 13/14

5913, 2-2 K.RFDYKDQQFLNLMEK.L 1320.0 1 24/56

6011, 2-2 K.DQQFLNLMEK.L 1079.8 1 13/18

6670, 2-2 K.EALIDLGEEFSGR.G 953.9 1 17/24

6682, 2-2 K.EALIDLGEEFSGR.G 1146.5 2 18/24

6874, 1-2 K.EALIDLGEEFSGR.G 1020.7 1 18/24

6995, 1 K.EALIDLGEEFSGR.G 852.8 1 18/24

89 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 2 isoform 1

precursor 69283.5 35493916

9 (9 0 0

0 0)

5354, 2-2 R.YHVPVVVVPEGSASDTHEQAILR.L 643.4 1 33/88

6285, 1-2 R.LQVTNVLSQPLTQATVK.L 882.9 1 19/32

6363, 1 R.LQVTNVLSQPLTQATVK.L 550.4 1 17/32

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7360, 2-2 R.LSKEETVLATVQALQTASHLSQQADLR.S

907.1 1 29/104

7674, 2-2 K.NPILWNVADVVIK.F 860.1 1 16/24

8050, 1 K.NPILWNVADVVIK.F 1069.3 1 19/24

8793, 1-2 R.SIVEEIEDLVAR.L 550.5 1 17/22

8829, 1-2 R.SIVEEIEDLVAR.L 724.4 2 14/22

8865, 1 R.SIVEEIEDLVAR.L 1416.2 1 17/22

90 non-specific lipid-transfer protein isoform 1 proprotein 58993.3 19923233

7 (7 0 0

0 0)

3566, 1-2 K.KLEEEGEQFVKK.I 1078.1 1 26/44

3910, 2-2 K.KLEEEGEQFVK.K 1362.9 1 16/20

3975, 1-2 K.KLEEEGEQFVK.K 1268.7 1 16/20

6600, 1 K.GHPLGATGLAQCAELCWQLR.G 776.6 1 22/38

7947, 1-2 R.QLIQGGVAECVLALGFEK.M 734.0 1 16/34

8063, 1 R.QLIQGGVAECVLALGFEK.M 1258.1 1 20/34

8613, 1-2 K.ADCTITMADSDFLALMTGK.M 810.6 1 17/36

91 ATP-binding cassette sub-family D member 3 isoform a 75475.5 4506341

7 (7 0 0

0 0)

5612, 2-2 R.IANPDQLLTQDVEK.F 1192.6 1 19/26

5619, 2-2 K.VGITLFTVSHR.K 640.5 2 15/20

6124, 2-2 K.EYLDNVQLGHILER.E 897.8 1 17/26

8087, 2-2 R.VLGELWPLFGGR.L 649.5 1 14/22

8354, 1-2 R.VLGELWPLFGGR.L 663.2 1 15/22

8470, 1 R.VLGELWPLFGGR.L 1048.7 1 18/22

8930, 1-2 R.EGGWDSVQDWMDVLSGGEK.Q 808.1 1 17/36

92 long-chain-fatty-acid--CoA ligase 3 80419.6 42794752

7 (6 0 1

0 0)

5375, 2-2 R.LLLCGGAPLSATTQR.F 893.0 1 20/28

5667, 1 R.LLLCGGAPLSATTQR.F 542.9 4 15/28

6634, 1-2 K.GIIVHTMAAVEALGAK.A 756.6 1 15/30

7258, 1 R.NLFILAYNYK.M 1810.7 1 16/18

7408, 2-2 K.VLSEAAISASLEKFEIPVK.I 539.8 3 16/36

7592, 1 R.LSPEPWTPETGLVTDAFK.L 694.0 1 21/34

7769, 1 K.VLSEAAISASLEKFEIPVK.I 842.8 1 18/36

93 PREDICTED: liver carboxylesterase 1-like 27540.5

34191633

5

7 (7 0 0

0 0)

5236, 1 R.NGNPNGEGLPHWPEYNQK.E 517.3 1 15/34

5352, 1-2 R.NFHTVPYMVGINK.Q 876.5 1 18/24

6984, 2-2 K.LKDKEVAFWTNLFAK.K 1570.9 1 19/28

7274, 1 K.LKDKEVAFWTNLFAK.K 1143.1 1 26/56

7864, 2-2 K.EVAFWTNLFAK.K 782.7 1 16/20

8151, 2-2 K.TVIGDHGDELFSVFGAPFLK.E 1133.1 1 18/38

8223, 1 K.EVAFWTNLFAK.K 837.7 1 16/20

94 glutamate dehydrogenase 2, mitochondrial precursor 61433.6 31377775

6 (6 0 0

0 0)

4148, 1-2 R.YSTDVSVDEVK.A 1022.5 1 16/20

5303, 1-2 K.CAVVDVPFGGAK.A 735.2 3 14/22

5741, 1-2 K.YNLGLDLR.T 1269.7 1 13/14

6153, 1 R.DDGSWEVIEGYR.A 1696.9 1 18/22

6284, 1-2 K.CIAVGESDGSIWNPDGIDPK.E 555.5 2 16/38

6357, 1 K.CIAVGESDGSIWNPDGIDPK.E 1595.3 1 26/38

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95 cytochrome P450 2D6 isoform 1 55730.1 40805836

6 (6 0 0

0 0)

5583, 1-2 R.VQQEIDDVIGQVR.R 1562.5 1 18/24

6270, 1-2 K.AVSNVIASLTCGR.R 1371.7 1 18/24

6952, 1-2 R.LLDLAQEGLKEESGFLR.E 546.3 1 16/32

8078, 1-2 K.AFLTQLDELLTEHR.M 1245.5 1 17/26

8082, 1-2 K.AFLTQLDELLTEHR.M 637.1 1 13/26

8274, 1 K.GTTLITNLSSVLKDEAVWEKPFR.F 715.2 1 28/88

96 17-beta-hydroxysteroid dehydrogenase type 6 35965.5 19743808

9 (9 0 0

0 0)

4898, 2-2 R.GQTSDRLETVTLDVTK.M 950.6 1 19/30

4914, 2-2 R.GQTSDRLETVTLDVTK.M 873.5 1 18/30

5030, 1-2 R.GQTSDRLETVTLDVTK.M 621.2 1 16/30

5877, 2-2 R.IVNVSSILGR.V 1250.5 1 16/18

6008, 1-2 K.ISIVEPGYFR.T 1092.8 1 16/18

6032, 1-2 R.IVNVSSILGR.V 1303.6 1 16/18

6089, 1 K.ISIVEPGYFR.T 1105.3 1 16/18

7798, 2-2 K.EGLLNCSTNLNLVTDCMEHALTSVHPR.T

1287.1 1 31/104

8637, 1-2 K.ETYGQQYFDALYNIMK.E 707.9 1 16/30

97 desmoplakin isoform I 331771.4 58530840

5 (5 0 0

0 0)

6304, 1-2 K.ITNLTQQLEQASIVK.K 1325.5 1 18/28

7098, 1 K.TIADLELHYQEFIR.N 540.0 1 15/26

7272, 1-2 K.SVEEVASEIQPFLR.G 519.0 1 13/26

7473, 2-2 R.GYFNEELSEILSDPSDDTK.G 509.4 1 13/36

7668, 1-2 R.TLELQGLINDLQR.E 703.1 1 15/24

98 trifunctional enzyme subunit beta, mitochondrial precursor 51294.1 4504327

6 (6 0 0

0 0)

4383, 2-2 R.LAAAFAVSR.L 1073.1 1 14/16

4709, 1-2 R.AALTGLLHR.T 936.1 1 14/16

5472, 2-2 K.DQLLLGPTYATPK.V 921.4 1 16/24

6129, 2-2 K.FNNWGGSLSLGHPFGATGCR.L 754.4 1 29/76

6300, 1-2 K.FNNWGGSLSLGHPFGATGCR.L 793.2 1 27/76

8562, 1 K.EVVDYIIFGTVIQEVK.T 650.4 1 17/30

99 general vesicular transport factor p115 107906.7 4505541

5 (5 0 0

0 0)

3508, 2-2 K.TLEQHDNIVTHYK.N 569.5 1 14/24

6659, 1 R.DSEQVAELKQELATLK.S 1202.7 1 21/30

6688, 1-2 K.VTNLHLMLQLVR.V 1249.9 1 17/22

7301, 2-2 R.QSEDLGSQFTEIFIK.Q 544.3 1 14/28

7482, 1 K.EQDDLLVLLADQDQK.I 781.1 1 17/28

100 ATP-dependent RNA helicase DDX3X isoform 2 73156.0

30117146

7

5 (5 0 0

0 0)

5186, 2-2 K.SPILVATAVAAR.G 1348.4 1 18/22

6047, 2-2 R.LEQELFSGGNTGINFEK.Y 667.8 1 18/32

6848, 2-2 R.VGNLGLATSFFNER.N 1081.9 1 18/26

6906, 2-2 K.HVINFDLPSDIEEYVHR.I 952.8 1 24/64

8495, 1 R.SFLLDLLNATGK.D 2175.9 1 19/22

101 serum paraoxonase/lactonase 3 39607.2 29788996

5 (5 0 0

0 0)

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4384, 2-2 K.GFCSANGITVSADQK.Y 832.3 1 16/28

4410, 1-2 K.YVYVADVAAK.N 741.0 1 11/18

5820, 1-2 K.LLNYNPEDPPGSEVLR.I 698.2 1 20/30

6245, 2-2 K.IFLMDLNEQNPR.A 1632.2 1 17/22

7068, 1-2 K.SVNDIVVLGPEQFYATR.D 512.2 1 17/32

102 cytochrome P450 3A7 57525.2

26229093

2

6 (6 0 0

0 0)

4698, 2-2 R.VLQNFSFKPCK.E 779.0 1 15/20

6684, 2-2 K.DNIDPYIYTPFGSGPR.N 889.1 1 17/30

6900, 1-2 K.DNIDPYIYTPFGSGPR.N 898.5 1 16/30

7148, 2-2 K.LKEMVPIIAQYGDVLVR.N 686.6 1 18/32

7751, 1-2 K.EMVPIIAQYGDVLVR.N 513.4 1 16/28

7768, 2-2 R.VDFLQLMIDSQNSK.D 1584.7 1 19/26

103 synaptic vesicle membrane protein VAT-1 homolog 41920.0 18379349

9 (9 0 0

0 0)

4787, 2-2 R.PAAPPAPGPGQLTLR.L 514.1 1 17/28

4796, 2-2 R.TVENVTVFGTASASK.H 935.8 1 18/28

4913, 1-2 R.TVENVTVFGTASASK.H 1016.6 1 18/28

5986, 2-2 R.CLVLTGFGGYDK.V 951.3 4 15/22

6142, 1-2 R.CLVLTGFGGYDK.V 799.3 3 14/22

6334, 2-2 K.VVTYGMANLLTGPK.R 1097.9 1 17/26

6538, 1-2 K.VVTYGMANLLTGPK.R 923.5 1 15/26

6750, 2-2 R.ACGLNFADLMAR.Q 1389.0 1 18/22

6956, 1-2 R.ACGLNFADLMAR.Q 1163.8 1 16/22

104 delta(24)-sterol reductase precursor 60100.9 13375618

6 (6 0 0

0 0)

5326, 1-2 K.LGCQDAFPEVYDK.I 1029.3 1 17/24

5696, 2-2 R.EGLEYIPLR.H 749.9 1 14/16

6363, 2-2 R.PGWLTVSLR.V 1020.1 1 14/16

6803, 2-2 K.LNSIGNYYKPWFFK.H 882.1 1 16/26

7023, 1-2 K.LNSIGNYYKPWFFK.H 663.0 1 14/26

7470, 2-2 R.YLFGWMVPPK.I 673.7 1 13/18

105 17-beta-hydroxysteroid dehydrogenase 13 isoform A 33655.2

21003211

0

8 (7 1 0

0 0)

4100, 1 R.KLGVTAHAYVVDCSNR.E 2044.2 1 30/60

4256, 1-2 K.LGVTAHAYVVDCSNR.E 1279.1 1 19/28

4469, 1 K.LGVTAHAYVVDCSNR.E 795.8 1 15/28

4906, 1 K.LGVTAHAYVVDCSNREEIYR.S 516.3 1 16/38

6003, 2-2 K.RQSILVLWDINKR.G 1624.9 1 28/48

6012, 2-2 K.RQSILVLWDINKR.G 785.2 1 17/24

6870, 1-2 R.QSILVLWDINKR.G 693.0 1 15/22

6956, 1 R.QSILVLWDINKR.G 990.6 1 18/22

106 histone H4 11367.3 4504301

6 (5 1 0

0 0)

4018, 2-2 R.DNIQGITKPAIR.R 738.3 1 15/22

4820, 2-2 R.ISGLIYEETR.G 1216.0 1 17/18

4916, 1-2 R.ISGLIYEETR.G 1214.7 1 17/18

6135, 2-2 R.KTVTAMDVVYALK.R 768.4 1 15/24

6294, 2-2 K.TVTAMDVVYALKR.Q 840.4 1 14/24

7005, 1-2 K.TVTAMDVVYALK.R 1260.1 1 19/22

107 UDP-glucuronosyltransferase 2B4 precursor 60512.1

14994450

9

7 (5 2 0

0 0)

Page 84: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

4703, 1-2 K.TVINDPLYKENAMK.L 569.6 1 16/26

5768, 2-2 K.FEVYPVSLTK.T 739.3 1 13/18

6202, 2-2 K.ADIWLIR.N 765.6 1 11/12

6224, 2-2 K.TILDELVQR.G 897.4 1 15/16

6398, 1-2 K.TILDELVQR.G 919.7 1 15/16

6485, 2-2 R.ANVIASALAKIPQK.V 650.7 1 15/26

6681, 1-2 R.ANVIASALAKIPQK.V 856.5 1 17/26

108 alcohol dehydrogenase 1B 39835.3 34577061

9 (9 0 0

0 0)

4258, 2-2 K.AKELGATECINPQDYK.K 1042.3 1 18/30

4355, 1-2 K.AKELGATECINPQDYK.K 980.4 1 21/30

6378, 1 K.VCLIGCGFSTGYGSAVNVAK.V 2888.7 1 30/38

7599, 2-2 K.VTPGSTCAVFGLGGVGLSAVMGCK.A

1578.3 1 28/46

7612, 2-2 K.VTPGSTCAVFGLGGVGLSAVMGCK.A

1144.2 1 28/46

7853, 1-2 K.VTPGSTCAVFGLGGVGLSAVMGCK.A

938.3 1 27/46

7869, 1-2 K.VTPGSTCAVFGLGGVGLSAVMGCK.A

2082.2 1 31/46

7989, 1 K.VTPGSTCAVFGLGGVGLSAVMGCK.A

1042.9 1 25/46

8272, 1 K.KFSLDALITHVLPFEK.I 1452.1 1 30/60

109 calpain-1 catalytic subunit 81889.4 12408656

4 (4 0 0

0 0)

6178, 1-2 R.LEICNLTPDALK.S 1106.7 3 15/22

6188, 1-2 R.KAPSDLYQIILK.A 806.4 1 15/22

6896, 1-2 R.NYPATFWVNPQFK.I 879.4 1 16/24

7271, 1 R.FRLPPGEYVVVPSTFEPNKEGDFVLR.F

1819.4 1 35/100

110 actin, cytoplasmic 1 41736.5 4501885

6 (6 0 0

0 0)

5248, 2-2 R.VAPEEHPVLLTEAPLNPK.A 615.9 1 18/34

6269, 1-2 K.DLYANTVLSGGTTM*YPGIADR.M 1620.0 1 23/40

6443, 2-2 R.TTGIVM*DSGDGVTHTVPIYEGYALPHAILR.L

1203.5 1 35/116

6652, 1-2 R.TTGIVM*DSGDGVTHTVPIYEGYALPHAILR.L

1079.4 1 33/116

6738, 1 R.TTGIVM*DSGDGVTHTVPIYEGYALPHAILR.L

1177.7 1 37/116

6924, 1-2 R.TTGIVMDSGDGVTHTVPIYEGYALPHAILR.L

1739.5 1 39/116

111 cytochrome P450 4A11 59347.5

15893724

2

4 (4 0 0

0 0)

3676, 2-2 K.CAFSHQGSIQVDR.N 1400.3 1 28/48

5348, 1 R.NAFHQNDTIYSLTSAGR.W 972.6 1 21/32

7449, 1 K.ALQQFPCPPSHWLFGHIQELQQDQELQR.I

586.2 1 33/108

8686, 1-2 R.NSQSYIQAISDLNNLVFSR.V 1083.3 1 22/36

112 sorbitol dehydrogenase 38324.3

15662757

1

5 (5 0 0

0 0)

Page 85: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5360, 1-2 R.MHSVGICGSDVHYWEYGR.I 926.7 1 20/34

6153, 2-2 K.AMGAAQVVVTDLSATR.L 1275.9 1 21/30

6340, 1-2 K.AMGAAQVVVTDLSATR.L 526.7 1 16/30

7625, 1 K.LPDNVTFEEGALIEPLSVGIHACR.R 1304.7 1 34/92

8793, 1 K.VLVCGAGPIGMVTLLVAK.A 509.2 1 15/34

113 perilipin-1 55990.0

22371820

3

9 (9 0 0

0 0)

5255, 2-2 R.LASGGADLALGSIEK.V 2064.4 1 22/28

5374, 1 R.LSTQFTAANELACR.G 1647.5 1 19/26

5402, 1-2 R.LASGGADLALGSIEK.V 2205.5 1 21/28

5534, 1 R.LASGGADLALGSIEK.V 1885.8 1 21/28

6747, 2-2 K.GLTLLDGDLPEQENVLQR.V 509.6 1 18/34

6963, 1-2 K.GLTLLDGDLPEQENVLQR.V 716.9 1 21/34

6972, 2-2 K.VLGAALAGCELAWGVAR.D 2072.2 1 23/32

7173, 1-2 K.VLGAALAGCELAWGVAR.D 1845.5 1 23/32

7280, 1 K.VLGAALAGCELAWGVAR.D 2265.0 1 24/32

114 myosin-Ib isoform 2 124949.7 44889481

6 (6 0 0

0 0)

4902, 1-2 K.AAHIFNEALVCHQIR.Y 1409.8 1 20/28

5357, 1-2 K.VSTTLNVAQAYYAR.D 563.9 1 15/26

7245, 2-2 R.IFLLTNNNLLLADQK.S 1443.9 1 18/28

7461, 1 R.NFYELSPHIFALSDEAYR.S 837.1 1 21/34

7486, 1-2 R.IFLLTNNNLLLADQK.S 1941.1 1 20/28

7487, 1-2 R.IFLLTNNNLLLADQK.S 1877.6 1 20/28

115 catenin alpha-1 100070.6 55770844

4 (4 0 0

0 0)

4271, 1-2 K.IAEQVASFQEEK.S 730.6 1 15/22

6581, 1-2 K.LIEVANLACSISNNEEGVK.L 1011.3 1 18/36

7370, 2-2 R.LLILADMADVYK.L 2181.7 1 20/22

9833, 1 R.VLTDAVDDITSIDDFLAVSENHILEDVNK.C

1047.3 1 32/112

116 elongation factor 1-alpha 2 50469.8 4503475

8 (8 0 0

0 0)

4442, 2-2 K.IGGIGTVPVGR.V 1159.3 1 18/20

4666, 2-2 K.THINIVVIGHVDSGK.S 994.7 1 16/28

4794, 1-2 K.THINIVVIGHVDSGK.S 1071.2 1 20/28

4956, 1 K.THINIVVIGHVDSGK.S 2255.0 1 31/56

4970, 1 K.THINIVVIGHVDSGK.S 1278.6 1 20/28

5672, 1-2 R.EHALLAYTLGVK.Q 1415.3 1 18/22

5769, 1 R.EHALLAYTLGVK.Q 1002.9 1 16/22

9434, 2-2 K.NMITGTSQADCAVLIVAAGVGEFEAGISK.N

1553.3 1 37/112

117 hemoglobin subunit delta 16055.4 4504351

5 (5 0 0

0 0)

3989, 1-2 K.VVAGVANALAHK.Y 1474.7 1 19/22

4244, 2-2 K.VVAGVANALAHKYH.- 1299.3 1 18/26

4331, 1-2 K.VVAGVANALAHKYH.- 1953.4 1 21/26

4661, 1-2 K.VNVDAVGGEALGR.L 1429.0 1 20/24

6716, 1 K.VLGAFSDGLAHLDNLK.G 1598.9 1 21/30

118 prohibitin-2 isoform 1 33296.1

22130758

4

7 (7 0 0

0 0)

5298, 1-2 K.FNASQLITQR.A 1535.3 1 17/18

Page 86: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5888, 2-2 K.LLLGAGAVAYGVR.E 2237.8 1 21/24

6131, 1 K.LLLGAGAVAYGVR.E 1882.6 1 19/24

6333, 2-2 R.IGGVQQDTILAEGLHFR.I 1225.1 1 28/64

6345, 2-2 R.IGGVQQDTILAEGLHFR.I 745.6 1 16/32

6531, 1-2 R.IGGVQQDTILAEGLHFR.I 1741.2 1 32/64

8079, 1 R.IYLTADNLVLNLQDESFTR.G 1003.9 1 20/36

119 alpha-mannosidase 2 131139.6 51477714

4 (4 0 0

0 0)

4926, 2-2 K.NKVEDSGIFTIK.N 685.0 1 14/22

5530, 2-2 R.LLAENNEIISNIR.D 1540.1 1 20/24

5806, 1 K.ILESASSNSHLADYVLYK.N 1214.3 1 22/34

7304, 1 K.FLSSSLYTALTEAR.R 1078.4 1 17/26

120 junction plakoglobin 81744.3 12056468

5 (5 0 0

0 0)

4563, 2-2 R.AGDKDDITEPAVCALR.H 814.2 1 17/30

5835, 1 R.NLALCPANHAPLQEAAVIPR.L 973.2 1 27/76

6216, 1-2 R.LVQNCLWTLR.N 705.7 4 13/18

8896, 1 K.TLVTQNSGVEALIHAILR.A 1203.2 1 20/34

8897, 1 K.TLVTQNSGVEALIHAILR.A 667.0 1 32/68

121 adipocyte plasma membrane-associated protein 46480.1 24308201

5 (5 0 0

0 0)

3411, 2-2 R.RPLRPQVVTDDDGQAPEAK.D 1269.3 1 29/72

4370, 2-2 K.LENGEIETIAR.F 898.3 1 16/20

4433, 2-2 R.LLEYDTVTR.E 835.9 1 15/16

4467, 1-2 K.LENGEIETIAR.F 1417.2 1 17/20

6552, 1-2 K.EPPLLLGVLHPNTK.L 718.1 1 16/26

122 cathepsin D preproprotein 44552.0 4503143

5 (5 0 0

0 0)

5604, 2-2 K.FDGILGM*AYPR.I 1167.8 1 17/20

6538, 2-2 K.FDGILGMAYPR.I 1228.9 1 17/20

7030, 1-2 K.AIGAVPLIQGEYMIPCEK.V 983.1 1 21/34

7037, 1-2 K.AIGAVPLIQGEYMIPCEK.V 1247.6 1 23/34

8013, 1-2 K.LVDQNIFSFYLSR.D 1103.3 1 18/24

123 UDP-glucuronosyltransferase 2B15 precursor 61036.0

11651729

9

5 (5 0 0

0 0)

4464, 2-2 K.GAALSVDIR.T 925.1 1 14/16

5330, 2-2 K.SSAIKLEVYPTSLTK.N 1248.7 1 19/28

5480, 1-2 K.SSAIKLEVYPTSLTK.N 1449.3 1 21/28

7162, 2-2 K.VLVWPTEYSHWINMK.T 571.5 1 16/28

8579, 1 R.TYWDFEFPRPFLPNVDFVGGLHCK.P

963.9 1 30/92

124 redox-regulatory protein PAMM isoform 1 precursor 25764.0

34492582

8

5 (5 0 0

0 0)

4984, 2-2 R.TEVKDFQPYFK.G 1060.0 1 15/20

7529, 2-2 K.VNLLSVLEAAK.M 860.5 1 16/20

7684, 2-2 K.SMLDQLGVPLYAVVK.E 939.5 1 20/28

7817, 2-2 R.LGVWYNFFR.A 583.3 1 13/16

7948, 1-2 K.SMLDQLGVPLYAVVK.E 525.1 2 13/28

125 retinol dehydrogenase 16 35673.2

15024722

6

5 (5 0 0

0 0)

5007, 1-2 K.VAM*IEPGYFK.T 834.5 1 14/18

5114, 2-2 K.TESVAAAAQWVK.E 1196.3 1 16/22

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5337, 2-2 R.DKYVFITGCDSGFGK.L 1410.8 1 19/28

5351, 2-2 R.DKYVFITGCDSGFGK.L 2907.7 1 32/56

7875, 2-2 R.DKGLWGLVNNAGISLPTAPNELLTK.Q

593.8 1 32/96

126 retinol dehydrogenase 10 38087.3 25282469

5 (5 0 0

0 0)

5397, 1-2 K.FGVVGFHESLSHELK.A 1392.8 1 19/28

6509, 2-2 R.LFALEFAR.R 746.2 1 13/14

6788, 1 R.LFALEFAR.R 722.2 1 13/14

7251, 1 K.EVGEVSVLVNNAGVVSGHHLLECPDELIER.T

755.2 1 32/116

7967, 2-2 K.SILPFEAVVCMYR.F 997.3 1 17/24

127 dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit

precursor 50701.3 20070197

6 (6 0 0

0 0)

5729, 2-2 K.NTLLIAGLQAR.N 1830.4 1 19/20

5770, 2-2 R.YSQTGNYELAVALSR.W 621.3 1 15/28

5853, 1-2 K.NTLLIAGLQAR.N 1849.3 1 18/20

6621, 2-2 R.TLVLLDNLNVR.E 1021.7 1 15/20

6932, 1 R.TLVLLDNLNVR.E 1120.0 1 16/20

7160, 2-2 K.LPDVYGVFQFK.V 937.0 1 15/20

128 betaine--homocysteine S-methyltransferase 1 44998.2

15726633

7

4 (4 0 0

0 0)

4928, 1 K.ISGQEVNEAACDIAR.Q 1099.8 1 20/28

5289, 1 K.AGPWTPEAAVEHPEAVR.Q 695.0 1 18/32

5877, 1 R.QVADEGDALVAGGVSQTPSYLSCK.S

605.3 1 30/92

6442, 1 K.AGASIIGVNCHFDPTISLK.T 577.6 1 14/36

129 cytochrome b5 isoform 1 15330.0 41281768

5 (5 0 0

0 0)

4137, 1-2 R.EQAGGDATENFEDVGHSTDAR.E 682.7 1 18/40

4425, 2-2 K.STWLILHHK.V 811.6 2 12/16

4526, 1-2 K.FLEEHPGGEEVLR.E 548.9 1 17/24

4979, 2-2 K.TFIIGELHPDDRPK.L 717.9 1 14/26

5103, 1-2 K.TFIIGELHPDDRPK.L 1665.3 1 20/26

130 eukaryotic initiation factor 4A-II 46402.0 83700235

5 (5 0 0

0 0)

4569, 1-2 K.LQAEAPHIVVGTPGR.V 1104.8 1 20/28

6086, 2-2 R.VLITTDLLAR.G 1282.8 1 17/18

6246, 1-2 R.VLITTDLLAR.G 1193.8 1 15/18

6671, 1-2 R.GFKDQIYEIFQK.L 1390.2 1 16/22

7574, 1-2 K.MFVLDEADEMLSR.G 2422.9 1 21/24

131 apoptosis-inducing factor 1, mitochondrial isoform 2 precursor 66294.3 22202629

5 (5 0 0

0 0)

5946, 2-2 K.ELWFSDDPNVTK.T 1004.7 1 15/22

6840, 2-2 R.ALGTEVIQLFPEK.G 635.9 2 13/24

7062, 1-2 R.ALGTEVIQLFPEK.G 1169.1 1 18/24

7091, 1-2 R.SNIWVAGDAACFYDIK.L 754.6 1 18/30

8855, 1-2 K.SITIIGGGFLGSELACALGR.K 545.2 1 16/38

132 arylacetamide deacetylase 45733.4 68299767

4 (4 0 0

0 0)

3612, 2-2 K.GHVYNNPNYGSSELAK.K 1508.7 1 22/30

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6208, 2-2 R.FWSEYFTTDR.S 942.5 1 16/18

7149, 2-2 R.LINQYIEWLK.E 1173.8 1 16/18

7901, 2-2 R.IGISGDSAGGNLAAAVTQQLLDDPDVK.I

700.7 1 20/52

133 myosin-Ic isoform c 117906.3

12449424

0

4 (4 0 0

0 0)

5922, 2-2 K.GDVVLQSDHVIETLTK.T 678.5 1 14/30

6065, 1-2 K.TLFATEDALEVR.R 734.8 1 16/22

6701, 2-2 R.LLQSNPVLEAFGNAK.T 856.5 1 17/28

9264, 1 K.MSLLQLVEILQSK.E 1201.0 1 17/24

134 prenylcysteine oxidase 1 precursor 56639.8

16679530

1

4 (4 0 0

0 0)

6434, 2-2 K.FLNEMIAPVMR.V 1112.2 1 15/20

6812, 2-2 K.IAIIGAGIGGTSAAYYLR.Q 1530.5 1 23/34

7199, 2-2 R.SDFYDIVLVATPLNRK.M 819.5 1 14/30

8146, 1 R.SDFYDIVLVATPLNR.K 782.9 1 16/28

135 coatomer subunit gamma 97717.7 11559929

5 (4 1 0

0 0)

5838, 1-2 R.ALQQYTLEPSEKPFDLK.S 1016.5 1 20/32

6136, 2-2 K.SSPEPVALTESETEYVIR.C 1058.7 1 20/34

6658, 2-2 R.ALCQITDSTMLQAIER.Y 650.0 1 14/30

6894, 1-2 R.ALCQITDSTMLQAIER.Y 944.1 1 16/30

7001, 1-2 R.QEIFQEQLAAVPEFR.G 693.0 1 16/28

136 cytochrome P450 2A6 precursor 56517.1

18933923

3

4 (4 0 0

0 0)

4707, 2-2 K.GYGVVFSNGER.A 1110.9 1 16/20

6070, 2-2 K.DKEFLSLLR.M 1072.7 1 15/16

6375, 2-2 R.FDYKDKEFLSLLR.M 1033.1 1 18/24

7691, 2-2 R.TVSNVISSIVFGDRFDYK.D 944.8 1 20/34

137 cytoplasmic dynein 1 heavy chain 1 532406.6 33350932

4 (4 0 0

0 0)

4344, 1-2 R.FTQDTQPHYIYSPR.E 531.1 1 18/26

6810, 1-2 R.QALVAIFTHLR.K 717.9 1 14/20

6964, 2-2 K.LNTQEIFDDWAR.K 551.4 1 12/22

7112, 2-2 R.MLSAVSQQVQCIQEALR.E 812.4 1 15/32

138 annexin A4 36084.8 4502105

4 (4 0 0

0 0)

4476, 2-2 K.GLGTDDNTLIR.V 884.7 1 14/20

4698, 1-2 R.NHLLHVFDEYKR.I 777.2 1 15/22

7295, 1-2 K.GAGTDEGCLIEILASR.T 514.3 1 16/30

7954, 1-2 K.GLGTDEDAIISVLAYR.N 1092.7 1 18/30

139 dynamin-1-like protein isoform 1 81876.7

17146091

4

4 (4 0 0

0 0)

5808, 2-2 R.RPLILQLVHVSQEDK.R 573.5 1 13/28

6120, 1-2 K.GHAVNLLDVPVPVAR.K 1104.5 1 18/28

6255, 2-2 K.ALQGASQIIAEIR.E 986.8 1 16/24

8616, 1-2 K.LHDAIVEVVTCLLR.K 1386.5 1 17/26

140 cytochrome P450 3A5 isoform 1 57108.3 4503231

6 (6 0 0

0 0)

5944, 2-2 K.DVEINGVFIPK.G 1073.5 1 15/20

6112, 1-2 K.DVEINGVFIPK.G 1059.0 1 15/20

6802, 2-2 K.DSIDPYIYTPFGTGPR.N 909.8 1 17/30

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7029, 1-2 K.DSIDPYIYTPFGTGPR.N 601.4 1 14/30

8316, 1-2 K.EMFPIIAQYGDVLVR.N 559.8 1 13/28

8848, 1-2 R.LGIPGPTPLPLLGNVLSYR.Q 575.7 1 19/36

141 ADP/ATP translocase 2 32852.0

15607145

9

4 (4 0 0

0 0)

4361, 1-2 K.QIFLGGVDKR.T 748.6 1 15/18

5552, 1-2 R.AAYFGIYDTAK.G 805.3 1 16/20

6342, 1-2 K.DFLAGGVAAAISK.T 712.0 1 19/24

6772, 1-2 K.EQGVLSFWR.G 628.8 1 13/16

142 protein transport protein Sec24A isoform 1 119748.6

11617478

0

9 (8 1 0

0 0)

5182, 1 R.VDNLSDEGALNISDR.T 991.5 1 18/28

6935, 1-2 K.TLETQSALGPALQAAFK.L 552.7 2 15/32

7822, 2-2 R.TYINPFVSFLDQR.R 889.0 1 17/24

8073, 2-2 R.TYINPFVSFLDQR.R 1003.9 1 17/24

8075, 1-2 R.TYINPFVSFLDQR.R 866.1 1 17/24

8309, 1-2 R.TYINPFVSFLDQR.R 1082.9 1 17/24

8440, 1 R.TYINPFVSFLDQR.R 1443.2 1 21/24

8898, 1-2 R.DALVNAVIDSLSAYR.S 1556.6 1 19/28

8970, 1 R.DALVNAVIDSLSAYR.S 1514.7 1 19/28

143 fructose-bisphosphate aldolase B 39472.8 40354205

5 (5 0 0

0 0)

5408, 1-2 R.IADQCPSSLAIQENANALAR.Y 2193.5 1 26/38

5518, 1 R.IADQCPSSLAIQENANALAR.Y 2314.1 1 26/38

5524, 1 R.IADQCPSSLAIQENANALAR.Y 2355.2 1 28/38

6392, 1 K.KYTPEQVAMATVTALHR.T 1520.8 1 31/64

6786, 1 R.YASICQQNGLVPIVEPEVIPDGDHDLEHCQYVTEK.V

993.1 1 36/136

144 peroxisomal multifunctional enzyme type 2 isoform 2 79685.9 4504505

4 (4 0 0

0 0)

4874, 1-2 R.VVLVTGAGAGLGR.A 2007.9 1 21/24

5171, 2-2 R.AYALAFAER.G 1053.4 1 13/16

6321, 2-2 R.GALVVVNDLGGDFK.G 1861.1 1 20/26

6509, 1-2 R.GALVVVNDLGGDFK.G 2024.8 1 21/26

145 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 precursor 68569.0 4506675

5 (5 0 0

0 0)

6935, 1-2 K.VACITEQVLTLVNKR.I 737.6 1 15/28

7118, 2-2 K.ISVIVETVYTHVLHPYPTQITQSEK.Q 805.3 1 29/96

7377, 1-2 K.ISVIVETVYTHVLHPYPTQITQSEK.Q 709.9 1 27/96

7754, 2-2 R.ATSFLLALEPELEAR.L 1026.8 1 20/28

8025, 1-2 R.ATSFLLALEPELEAR.L 722.6 1 17/28

146 aldehyde oxidase 147916.9 71773480

3 (3 0 0

0 0)

7132, 2-2 K.TGIIAAVTAFAANK.H 1346.0 1 19/26

8244, 1 R.GFGFPQAALITESCITEVAAK.C 1032.1 1 22/40

9219, 1 K.VFCVGQLVCAVLADSEVQAK.R 1203.1 1 22/38

147 membrane-associated progesterone receptor component 1 21671.0 5729875

4 (4 0 0

0 0)

4251, 1-2 R.GDQPAASGDSDDDEPPPLPR.L 794.4 1 20/38

5561, 2-2 R.KFYGPEGPYGVFAGR.D 1708.5 1 22/28

5716, 1-2 R.KFYGPEGPYGVFAGR.D 1540.3 1 20/28

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6312, 1-2 K.FYGPEGPYGVFAGR.D 1256.1 1 21/26

148 tripeptidyl-peptidase 1 preproprotein 61247.6 5729770

4 (4 0 0

0 0)

4599, 2-2 R.LFGGNFAHQASVAR.V 1499.9 1 20/26

6108, 1-2 R.ILSGRPPLGFLNPR.L 808.6 1 18/26

6129, 1 R.ILSGRPPLGFLNPR.L 530.7 1 16/26

9308, 1 R.VPIPWVSGTSASTPVFGGILSLINEHR.I

2364.2 1 38/104

149 exportin-1 123385.0 4507943

3 (3 0 0

0 0)

4450, 2-2 K.NVDILKDPETVK.Q 530.2 1 14/22

6174, 2-2 K.EFAGEDTSDLFLEER.E 728.7 1 17/28

8603, 1 K.LDINLLDNVVNCLYHGEGAQQR.M 859.4 1 30/84

150 hydroxymethylglutaryl-CoA synthase, mitochondrial isoform 1 precursor 56635.1 5031751

4 (4 0 0

0 0)

5933, 2-2 R.PTGGAGAVAMLIGPK.A 1939.0 1 22/28

8752, 1 K.QAGSDRPFTLDDLQYMIFHTPFCK.M

779.1 1 25/92

8867, 1-2 R.IGAFSYGSGLAASFFSFR.V 539.3 1 14/34

8924, 1 R.IGAFSYGSGLAASFFSFR.V 1146.0 1 20/34

151 AP-2 complex subunit beta isoform b 104551.9 4557469

5 (5 0 0

0 0)

4284, 1-2 R.LSHANSAVVLSAVK.V 1018.7 1 18/26

7437, 2-2 R.LASQANIAQVLAELK.E 1625.2 1 21/28

7678, 1-2 R.LASQANIAQVLAELK.E 1390.3 1 21/28

7816, 1 R.LASQANIAQVLAELK.E 1513.8 1 21/28

8648, 1 K.KPSETQELVQQVLSLATQDSDNPDLR.D

1344.8 1 34/100

152 aldehyde dehydrogenase, mitochondrial isoform 1 precursor 56380.9 25777732

4 (4 0 0

0 0)

6218, 1-2 R.ANNSTYGLAAAVFTK.D 656.7 1 15/28

7191, 1-2 R.TFVQEDIYDEFVER.S 1136.7 1 17/26

8124, 1-2 K.VAEQTPLTALYVANLIK.E 994.7 1 23/32

8230, 1 K.VAEQTPLTALYVANLIK.E 704.6 1 19/32

153 hemoglobin subunit alpha 15257.4 4504345

6 (6 0 0

0 0)

3932, 1-2 K.VGAHAGEYGAEALER.M 1170.6 1 21/28

3938, 1-2 K.VGAHAGEYGAEALER.M 1134.4 2 26/56

6010, 1-2 K.FLASVSTVLTSK.Y 1218.5 1 17/22

6068, 1 K.FLASVSTVLTSK.Y 1272.6 1 18/22

7570, 1-2 K.VADALTNAVAHVDDMPNALSALSDLHAHK.L

999.9 1 27/112

7692, 1 K.VADALTNAVAHVDDMPNALSALSDLHAHK.L

1160.6 1 32/112

154 cytochrome P450 1A2 58407.1 73915100

4 (4 0 0

0 0)

4594, 2-2 R.FLTADGTAINKPLSEK.M 2206.7 1 24/30

4882, 2-2 R.YGDVLQIR.I 968.5 1 13/14

5306, 2-2 R.IGSTPVLVLSR.L 965.8 1 17/20

5456, 1-2 R.IGSTPVLVLSR.L 800.7 1 16/20

155 reticulon-4 isoform A 129930.3 24431935 5 (5 0 0

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0 0)

3902, 2-2 K.YSNSALGHVNCTIK.E 1498.4 1 21/26

4443, 2-2 R.HQAQIDHYLGLANK.N 1757.6 1 20/26

4565, 1-2 R.HQAQIDHYLGLANK.N 1265.9 1 17/26

6753, 1-2 R.AYLESEVAISEELVQK.Y 851.6 1 16/30

6896, 1 R.AYLESEVAISEELVQK.Y 630.8 1 20/30

156 carnitine O-palmitoyltransferase 2, mitochondrial precursor 73776.6 4503023

3 (3 0 0

0 0)

4481, 2-2 K.ELHEQLVALDK.Q 935.7 1 15/20

8255, 1 K.YILSDSSPAPEFPLAYLTSENR.D 734.0 1 21/42

9484, 1 K.LSPDAVAQLAFQMAFLR.Q 1361.3 1 18/32

157 myosin-10 228996.6 41406064

3 (3 0 0

0 0)

7192, 1-2 R.QLLQANPILESFGNAK.T 828.1 1 17/30

7251, 1-2 K.VVSSVLQFGNISFK.K 937.7 1 17/26

7370, 1 K.LQQLFNHTMFILEQEEYQR.E 1511.6 1 30/72

158 AP-2 complex subunit alpha-1 isoform 2 105360.8 19913416

3 (3 0 0

0 0)

4733, 1-2 R.VLQIVTNRDDVQGYAAK.T 691.3 1 18/32

6899, 1-2 R.GLAVFISDIR.N 1447.9 1 16/18

8210, 1-2 K.VGGYILGEFGNLIAGDPR.S 1275.5 1 20/34

159 heterogeneous nuclear ribonucleoprotein K isoform a 51027.9 14165437

4 (4 0 0

0 0)

5915, 1-2 R.LLIHQSLAGGIIGVK.G 664.0 1 16/28

5973, 1 R.LLIHQSLAGGIIGVK.G 1255.5 1 18/28

7392, 1-2 K.IILDLISESPIK.G 1391.9 1 16/22

7978, 1 K.IDEPLEGSEDRIITITGTQDQIQNAQYLLQNSVK.Q

768.0 1 32/132

160 ras-related protein Rab-14 23896.8 19923483

5 (5 0 0

0 0)

4895, 2-2 K.IYQNIQDGSLDLNAAESGVQHKPSAPQGGR.L

988.4 1 32/116

5040, 1-2 K.IYQNIQDGSLDLNAAESGVQHKPSAPQGGR.L

1216.2 1 38/116

5194, 1 K.IYQNIQDGSLDLNAAESGVQHKPSAPQGGR.L

926.8 1 34/116

6232, 1-2 K.TGENVEDAFLEAAK.K 677.6 1 16/26

6752, 1-2 R.NLTNPNTVIILIGNK.A 685.6 1 17/28

161 prohibitin 29803.9 4505773

3 (3 0 0

0 0)

5032, 2-2 R.IFTSIGEDYDER.V 627.7 1 14/22

7895, 1-2 K.AAELIANSLATAGDGLIELR.K 1113.5 1 21/38

8658, 1 R.AATFGLILDDVSLTHLTFGK.E 762.2 1 19/38

162 estradiol 17-beta-dehydrogenase 12 34323.9 7705855

5 (5 0 0

0 0)

6512, 1-2 R.TIAVDFASEDIYDKIK.T 1060.8 1 18/30

7361, 2-2 K.TFVDFFSQCLHEEYR.S 1223.0 1 18/28

7707, 2-2 K.GVFVQSVLPYFVATK.L 509.6 1 16/28

7710, 2-2 K.GVFVQSVLPYFVATK.L 532.6 1 18/28

7744, 1 K.TFVDFFSQCLHEEYR.S 1384.1 1 19/28

163 hemoglobin subunit beta 15998.3 4504349

5 (4 1 0

0 0)

Page 92: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5931, 1-2 K.GTFATLSELHCDKLHVDPENFR.L 561.4 1 26/84

6893, 2-2 R.FFESFGDLSTPDAVMGNPK.V 874.9 1 20/36

6910, 2-2 R.FFESFGDLSTPDAVMGNPK.V 521.0 1 16/36

7109, 1-2 R.FFESFGDLSTPDAVMGNPK.V 1089.7 1 22/36

8160, 1-2 R.LLGNVLVCVLAHHFGK.E 699.0 1 15/30

164 glutamate dehydrogenase 1, mitochondrial precursor 61397.5 4885281

4 (4 0 0

0 0)

5601, 1 K.HGGTIPIVPTAEFQDR.I 537.4 1 18/30

5858, 1-2 R.GASIVEDKLVEDLR.T 1377.4 1 19/26

6611, 1-2 K.AKPYEGSILEADCDILIPAASEK.Q 504.5 1 19/44

6690, 1 K.AKPYEGSILEADCDILIPAASEK.Q 818.2 1 22/44

165 SEC14-like protein 2 isoform 1 46145.0 7110715

7 (7 0 0

0 0)

4166, 2-2 K.INYGGDIPR.K 719.6 1 13/16

4245, 1-2 K.INYGGDIPR.K 687.0 1 13/16

4785, 2-2 R.FDNTYSFIHAK.K 1325.1 1 17/20

4904, 1-2 R.FDNTYSFIHAK.K 1219.8 1 16/20

4906, 1-2 R.FDNTYSFIHAK.K 1258.1 1 17/20

8738, 1-2 K.LFPVAYNLIKPFLSEDTR.K 515.4 1 15/34

8829, 1 K.LFPVAYNLIKPFLSEDTR.K 701.8 1 19/34

166 40S ribosomal protein SA 32853.8 59859885

4 (4 0 0

0 0)

3965, 2-2 K.FAAATGATPIAGR.F 1490.5 1 20/24

4040, 1-2 K.FAAATGATPIAGR.F 1712.7 1 21/24

4936, 1-2 R.YVDIAIPCNNK.G 764.8 1 13/20

6944, 2-2 R.ADHQPLTEASYVNLPTIALCNTDSPLR.Y

675.7 1 28/104

167 guanine nucleotide-binding protein subunit beta-2-like 1 35076.5 5174447

5 (5 0 0

0 0)

4978, 1 K.YTVQDESHSEWVSCVR.F 588.1 1 15/30

5658, 2-2 K.IIVDELKQEVISTSSK.A 1049.8 1 20/30

5850, 2-2 R.FSPNSSNPIIVSCGWDK.L 723.3 1 17/32

6014, 1-2 R.FSPNSSNPIIVSCGWDK.L 678.3 1 18/32

6095, 1 R.FSPNSSNPIIVSCGWDK.L 539.6 1 16/32

168 carbamoyl-phosphate synthase [ammonia], mitochondrial isoform a precursor 165648.8

16979091

5

4 (3 1 0

0 0)

5537, 1 R.GAEVHLVPWNHDFTK.M 1549.2 1 27/56

6148, 2-2 K.VSGLLVLDYSK.D 1638.6 1 18/20

6316, 1-2 K.VSGLLVLDYSK.D 692.8 4 15/20

7912, 1 K.MEYDGILIAGGPGNPALAEPLIQNVR.K

1146.7 1 23/50

169 60S ribosomal protein L4 47697.0 16579885

4 (4 0 0

0 0)

5220, 1-2 R.KLDELYGTWR.K 912.7 1 13/18

5908, 2-2 K.APIRPDIVNFVHTNLR.K 943.3 1 24/60

6069, 1-2 K.APIRPDIVNFVHTNLR.K 802.5 1 23/60

6906, 1-2 R.FCIWTESAFR.K 1359.1 1 16/18

170 sodium/potassium-transporting ATPase subunit alpha-1 isoform c 112999.5

23768110

9

3 (3 0 0

0 0)

3272, 1-2 K.VDNSSLTGESEPQTR.S 687.5 1 17/28

5021, 2-2 R.SPDFTNENPLETR.N 1417.0 2 18/24

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6140, 1-2 K.GVGIISEGNETVEDIAAR.L 586.0 1 16/34

171 cytochrome b-c1 complex subunit 2, mitochondrial precursor 48442.7 50592988

3 (3 0 0

0 0)

3827, 2-2 K.TIAQGNLSNTDVQAAK.N 1678.5 1 23/30

5649, 1 R.NALANPLYCPDYR.I 752.6 1 17/24

6111, 1 R.MALIGLGVSHPVLK.Q 1069.1 2 17/26

172 phosphoglucomutase-1 isoform 1 61448.7 21361621

5 (4 1 0

0 0)

4168, 2-2 R.YDYEEVEAEGANK.M 1664.7 1 18/24

4259, 1-2 R.YDYEEVEAEGANK.M 1939.5 1 19/24

4791, 1-2 R.LYIDSYEKDVAK.I 1620.1 1 18/22

5187, 1 K.FNISNGGPAPEAITDK.I 1028.8 2 19/30

5265, 1 K.FNISNGGPAPEAITDK.I 969.1 1 21/30

173 corticosteroid 11-beta-dehydrogenase isozyme 1 32400.8 5031765

6 (6 0 0

0 0)

3870, 2-2 K.AVSGIVHMQAAPK.E 1148.4 1 17/24

3926, 1-2 K.AVSGIVHMQAAPK.E 1367.5 1 18/24

7234, 1-2 K.FALDGFFSSIRK.E 1138.9 1 17/22

7761, 2-2 K.FALDGFFSSIR.K 1363.9 1 17/20

8028, 1-2 K.FALDGFFSSIR.K 1267.7 1 17/20

8133, 1 K.FALDGFFSSIR.K 1342.3 1 17/20

174 translational activator GCN1 292707.0 54607053

4 (3 1 0

0 0)

6430, 2-2 R.LLTWVIGTGSPR.L 782.1 1 14/22

8619, 1-2 K.VLPLEALVTDAGEVTEAGK.A 756.0 1 17/36

9338, 1 K.AALLETLSLLLAK.V 1077.3 2 16/24

9471, 1-2 K.AALLETLSLLLAK.V 1285.2 1 17/24

175 phosphate carrier protein, mitochondrial isoform b precursor 39958.5 47132595

3 (3 0 0

0 0)

3951, 2-2 R.IQTQPGYANTLR.D 576.3 1 15/22

7115, 2-2 K.VLYSNMLGEENTYLWR.T 717.5 1 17/30

7757, 1-2 K.GWAPTFLGYSMQGLCK.F 835.5 1 18/30

176 transitional endoplasmic reticulum ATPase 89321.3 6005942

4 (4 0 0

0 0)

5374, 2-2 R.LGDVISIQPCPDVK.Y 538.4 3 14/26

5574, 1 K.AIANECQANFISIK.G 652.8 1 17/26

7319, 1-2 R.LDQLIYIPLPDEK.S 696.4 1 14/24

7460, 1 R.LDQLIYIPLPDEK.S 976.7 1 16/24

177 carnitine O-palmitoyltransferase 1, liver isoform isoform 1 88367.1 73623030

3 (3 0 0

0 0)

4745, 2-2 K.GDINPNIPYPTR.L 839.2 1 17/22

5439, 2-2 R.LAALTAGDRVPWAR.C 615.0 2 13/26

5542, 2-2 R.AGNAIHAILLYR.R 1065.9 1 17/22

178 exportin-2 110415.8 29029559

3 (3 0 0

0 0)

7710, 1 R.GSNTIASAAADKIPGLLGVFQK.L 705.4 1 19/42

8081, 1-2 K.LLQTDDEEEAGLLELLK.S 1059.5 1 18/32

8392, 1 K.YGALALQEIFDGIQPK.M 1111.7 1 19/30

179 cytosolic 10-formyltetrahydrofolate dehydrogenase 98828.6 21614513

3 (3 0 0

0 0)

5870, 1 K.GVVNVLPGSGSLVGQR.L 515.2 1 17/30

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6069, 1 R.LFVEDSIHDEFVR.R 796.0 1 14/24

6470, 1 K.VLEVEDSTDFFK.S 2127.5 1 20/22

180 filamin-B isoform 2 278160.3

10599051

4

4 (4 0 0

0 0)

5660, 1-2 R.IGNLQTDLSDGLR.L 684.4 1 14/24

5730, 1-2 R.GAGIGGLGITVEGPSESK.I 546.5 2 15/34

5842, 1 R.GAGIGGLGITVEGPSESK.I 619.6 1 19/34

8393, 1 R.LIALLEVLSQK.R 1061.8 1 17/20

181 microsomal triglyceride transfer protein large subunit precursor 99350.6

15328540

8

4 (4 0 0

0 0)

5830, 1 K.LTYSTEVLLDR.G 994.9 1 16/20

7659, 1-2 R.NFLAFIQHLR.T 1266.8 1 15/18

7748, 1 R.NFLAFIQHLR.T 1138.6 1 15/18

7761, 1 R.SASTYSLDILYSGSGILR.R 626.2 1 15/34

182 abhydrolase domain-containing protein 14B 22345.5 14249382

4 (4 0 0

0 0)

5450, 1-2 R.AVAIDLPGLGHSK.E 1019.6 1 18/24

6179, 2-2 R.FSVLLLHGIR.F 1053.6 1 15/18

6328, 2-2 R.EGTIQVQGQALFFR.E 661.8 1 13/26

6345, 1-2 R.FSVLLLHGIR.F 1122.6 1 15/18

183 cytochrome P450 2C18 isoform 1 precursor 55710.4 13699816

5 (5 0 0

0 0)

3627, 2-2 R.SIEDRVQEEAR.C 1040.6 1 15/20

3692, 1-2 R.SIEDRVQEEAR.C 938.2 1 14/20

7458, 2-2 R.YGLLLLLK.Y 981.9 1 12/14

7586, 2-2 R.DFIDCFLIK.M 1089.7 2 13/16

7695, 1-2 R.YGLLLLLK.Y 971.3 1 12/14

184 tricarboxylate transport protein, mitochondrial precursor 34012.4 21389315

3 (3 0 0

0 0)

5372, 1-2 K.GTYQGLTATVLK.Q 921.9 1 16/22

6867, 1-2 R.GLSSLLYGSIPK.A 1466.7 1 16/22

7618, 1-2 R.FGMFEFLSNHMR.D 626.8 1 13/22

185 alcohol dehydrogenase 1C 39867.4 4501933

6 (2 4 0

0 0)

5554, 1 K.KPFSIEEVEVAPPK.A 692.9 1 17/26

6240, 2-2 K.VCLIGCGFSTGYGSAVKVAK.V 1868.7 2 23/38

6257, 2-2 K.VCLIGCGFSTGYGSAVKVAK.V 2332.0 2 26/38

6440, 1-2 K.VCLIGCGFSTGYGSAVKVAK.V 2270.9 2 25/38

6648, 1 K.VCLIGCGFSTGYGSAVKVAK.V 941.5 2 20/38

9056, 1 K.KFSLDALITNILPFEK.I 2453.3 1 26/30

186 histone H2B type 1-C/E/F/G/I 13906.0 4504271

7 (5 2 0

0 0)

7892, 2-2 K.AM*GIMNSFVNDIFER.I 1667.2 1 21/28

7892, 2-2 K.AMGIM*NSFVNDIFER.I 968.5 2 17/28

8168, 1-2 K.AM*GIMNSFVNDIFER.I 1813.1 1 21/28

8168, 1-2 K.AMGIM*NSFVNDIFER.I 1203.8 2 18/28

8330, 2-2 K.AMGIMNSFVNDIFER.I 1041.3 1 18/28

8590, 1-2 K.AMGIMNSFVNDIFER.I 1790.4 1 21/28

8612, 1-2 K.AMGIMNSFVNDIFER.I 1424.1 1 20/28

187 ras-related protein Rab-7a 23489.6 34147513

5 (4 1 0

0 0)

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6190, 2-2 R.LVTMQIWDTAGQER.F 1783.1 1 22/26

6358, 1-2 R.LVTMQIWDTAGQER.F 1287.7 1 19/26

6891, 2-2 K.EAINVEQAFQTIAR.N 1027.2 1 17/26

7097, 1-2 K.EAINVEQAFQTIAR.N 1073.8 1 18/26

7268, 1-2 R.DPENFPFVVLGNK.I 593.5 1 14/24

188 UDP-glucuronosyltransferase 2A2 60771.7

33360924

5

4 (2 2 0

0 0)

6921, 2-2 K.ANLIASALAQIPQK.V 560.1 1 16/26

7161, 1-2 K.ANLIASALAQIPQK.V 1619.8 1 21/26

8092, 1-2 R.AVFWIEFVM*R.H 1003.9 1 13/18

9034, 1 R.AVFWIEFVMR.H 1206.7 1 14/18

189 cytochrome P450 4F11 precursor 60145.5

19308317

8

6 (4 2 0

0 0)

7316, 1-2 R.SPLAFIPFSAGPR.N 833.0 1 18/24

7424, 1 R.SPLAFIPFSAGPR.N 611.8 1 16/24

7511, 2-2 K.VVLALTLLHFR.I 807.8 2 15/20

7758, 1-2 K.VVLALTLLHFR.I 1175.1 2 16/20

7962, 2-2 K.TLDFIDVLLLSKDEDGK.E 980.5 1 17/32

8214, 1-2 K.TLDFIDVLLLSKDEDGK.E 794.9 1 16/32

190 vitronectin precursor 54305.3 88853069

3 (2 1 0

0 0)

5304, 2-2 R.FEDGVLDPDYPR.N 939.8 2 15/22

6969, 2-2 R.SIAQYWLGCPAPGHL.- 838.6 1 15/28

7644, 2-2 R.DVWGIEGPIDAAFTR.I 1000.0 1 18/28

191 voltage-dependent anion-selective channel protein 3 isoform 2 30789.6

20887946

5

5 (3 2 0

0 0)

4974, 2-2 K.VCNYGLTFTQK.W 1301.6 1 17/20

5112, 1-2 K.VCNYGLTFTQK.W 1100.5 1 16/20

5937, 1-2 K.VNNASLIGLGYTQTLRPGVK.L 1217.1 1 22/38

6648, 2-2 K.LTLSALIDGK.N 869.5 1 15/18

6851, 1-2 K.LTLSALIDGK.N 938.2 1 14/18

192 cytochrome P450 2A13 56687.2 13699809

4 (3 1 0

0 0)

4622, 2-2 R.RFSIATLR.G 1056.3 1 13/14

6375, 2-2 R.FDYEDKEFLSLLR.M 1033.1 1 18/24

7784, 2-2 R.DFIDSFLIR.M 946.5 2 13/16

8036, 1-2 R.DFIDSFLIR.M 837.1 2 13/16

193 UDP-glucuronosyltransferase 2B28 isoform 2 precursor 38743.0

33303381

1

3 (2 0 1

0 0)

4901, 2-2 K.LEVYPTSLTK.T 636.2 1 14/18

6453, 1 K.KWDQFYSEVLGR.P 518.4 1 15/22

7161, 1-2 R.ANVIATALAKIPQK.I 760.4 2 16/26

194 calcium-binding mitochondrial carrier protein Aralar1 74761.4 21361103

4 (3 0 0

1 0)

5182, 1-2 R.AGQTTYSGVIDCFRK.I 1150.5 1 19/28

6707, 2-2 R.YEGFFGLYR.G 909.7 1 15/16

6916, 1-2 R.YEGFFGLYR.G 821.2 1 15/16

7438, 1-2 R.DIPFSAIYFPVYAHCK.L 528.8 2 14/30

195 enoyl-CoA hydratase, mitochondrial precursor 31387.2

19409732

3

4 (4 0 0

0 0)

8642, 1-2 K.ALNALCDGLIDELNQALK.T 692.1 1 16/34

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8732, 1 K.ALNALCDGLIDELNQALK.T 1585.1 1 23/34

8736, 1 K.ALNALCDGLIDELNQALK.T 2049.9 1 25/34

9158, 1 K.ICPVETLVEEAIQCAEK.I 695.8 1 15/32

196 rab GDP dissociation inhibitor alpha 50582.4 4503971

2 (2 0 0

0 0)

5724, 2-2 R.FQLLEGPPESM*GRGR.D 537.8 1 15/28

8709, 1 R.KFDLGQDVIDFTGHALALYR.T 2649.8 1 37/76

197 protein disulfide-isomerase A3 precursor 56782.0 21361657

2 (2 0 0

0 0)

7407, 1-2 R.ELSDFISYLQR.E 535.3 1 14/20

8486, 1 K.FISDKDASIVGFFDDSFSEAHSEFLK.A

2906.1 1 40/100

198 glyceraldehyde-3-phosphate dehydrogenase 36053.0 7669492

2 (2 0 0

0 0)

5349, 1-2 R.GALQNIIPASTGAAK.A 597.0 1 16/28

8776, 1 K.VIHDNFGIVEGLMTTVHAITATQK.T 1098.5 1 32/92

199 alcohol dehydrogenase [NADP+] 36572.8 24497577

2 (2 0 0

0 0)

5979, 1 R.HIDCAAIYGNEPEIGEALKEDVGPGK.A

1445.3 1 36/100

8346, 1 R.AWRDPDEPVLLEEPVVLALAEK.Y 2061.6 1 37/84

200 apolipoprotein E precursor 36153.8 4557325

2 (2 0 0

0 0)

3680, 2-2 K.VQAAVGTSAAPVPSDNH.- 567.8 1 16/32

5044, 2-2 K.AYKSELEEQLTPVAEETR.A 1918.0 1 25/34

201 mitochondrial carrier homolog 2 33330.7 7657347

4 (4 0 0

0 0)

3621, 2-2 K.VLQHYQESDKGEELGPGNVQK.E 2162.8 1 36/80

3711, 1-2 K.VLQHYQESDKGEELGPGNVQK.E 1185.3 1 29/80

6030, 2-2 R.SAATLITHPFHVITLR.S 1366.4 1 30/60

6198, 1-2 R.SAATLITHPFHVITLR.S 554.6 1 18/30

202 fibrinogen gamma chain isoform gamma-A precursor 49496.2 70906437

2 (2 0 0

0 0)

4868, 1 K.VAQLEAQCQEPCKDTVQIHDITGK.D

828.9 1 28/92

5500, 2-2 K.QSGLYFIKPLK.A 631.9 2 13/20

203 UBX domain-containing protein 4 56777.2 24307965

2 (2 0 0

0 0)

4107, 1 K.SDTATGGESAGHATSSQEPSGCSDQRPAEDLNIR.V

829.0 1 32/132

7821, 2-2 -.MLWFQGAIPAAIATAK.R 516.0 1 13/30

204 ATP-binding cassette sub-family A member 6 184284.2 27436953

2 (2 0 0

0 0)

5978, 2-2 K.LPVADVYPLSQTFHK.L 753.7 1 17/28

6401, 2-2 R.TATALTTSILDEKPVIIASCLHK.E 852.6 1 27/88

205 histone H2A type 1-D 14107.4 10800130

4 (4 0 0

0 0)

5084, 2-2 R.AGLQFPVGR.V 805.1 1 14/16

5222, 1-2 R.AGLQFPVGR.V 830.3 1 14/16

7737, 2-2 K.VTIAQGGVLPNIQAVLLPK.K 954.7 1 23/36

8151, 1 K.VTIAQGGVLPNIQAVLLPK.K 1282.0 1 31/72

206 estradiol 17-beta-dehydrogenase 2 42784.9 4504503 4 (4 0 0

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0 0)

4784, 2-2 K.AVLVTGGDCGLGHALCK.Y 1767.0 1 24/32

4925, 1-2 K.AVLVTGGDCGLGHALCK.Y 1355.2 1 21/32

8430, 1-2 R.NFLLLINSLASK.D 2724.9 1 20/22

8501, 1 R.NFLLLINSLASK.D 2493.5 1 19/22

207 dehydrogenase/reductase SDR family member on chromosome X precursor 36442.9

19380485

0

2 (2 0 0

0 0)

5092, 1 R.VAIVTGGTDGIGYSTAK.H 1588.9 1 22/32

9356, 1 R.VYAVGAAVILAQLLR.R 1594.3 1 19/28

208 UDP-glucuronosyltransferase 1-7 precursor 59818.5 41282213

3 (3 0 0

0 0)

8937, 1-2 K.GAGVTLNVLEM*TSEDLENALK.A 1168.7 1 21/40

9215, 1 K.GAGVTLNVLEMTSEDLENALK.A 738.1 1 26/80

9242, 1-2 K.GAGVTLNVLEMTSEDLENALK.A 1275.3 1 21/40

209 7-dehydrocholesterol reductase 54489.1

11994311

2

4 (4 0 0

0 0)

6123, 2-2 K.FLPGYVGGIQEGAVTPAGVVNK.Y 1022.6 1 24/42

6302, 1-2 K.FLPGYVGGIQEGAVTPAGVVNK.Y 895.3 1 22/42

6940, 2-2 K.LLVSGFWGVAR.H 955.1 1 15/20

7169, 1-2 K.LLVSGFWGVAR.H 1045.1 1 16/20

210 protein-glutamine gamma-glutamyltransferase 2 isoform a 77328.4 39777597

2 (2 0 0

0 0)

7864, 1 K.YLLNLNLEPFSEK.S 1565.3 1 19/24

8128, 1 R.ALLVEPVINSYLLAER.D 1060.4 1 23/30

211 phosphatidylinositol-binding clathrin assembly protein isoform 1 70754.1 56788366

2 (2 0 0

0 0)

7936, 1-2 R.ATTLSNAVSSLASTGLSLTK.V 1562.4 1 23/38

8130, 1 R.NTLFNLSNFLDK.S 736.0 1 15/22

212 2,4-dienoyl-CoA reductase, mitochondrial precursor 36067.5 4503301

2 (2 0 0

0 0)

5715, 1 K.VAGHPNIVINNAAGNFISPTER.L 724.6 1 26/84

7196, 2-2 K.EQWDTIEELIR.K 889.4 1 14/20

213 SEC14-like protein 3 46047.9 27923592

2 (2 0 0

0 0)

7072, 1 R.GSSHQVEYEILFPGCVLR.W 1469.1 1 23/34

8226, 1-2 K.FRENVQDVLPALPNPDDYFLLR.W 951.1 1 32/84

214 UDP-glucuronosyltransferase 1-4 precursor 60024.7 6005930

4 (4 0 0

0 0)

5282, 2-2 R.CCVELLHNEALIR.H 1522.7 1 21/24

5430, 1-2 R.CCVELLHNEALIR.H 1619.6 1 21/24

5561, 1 R.CCVELLHNEALIR.H 1002.0 1 17/24

7587, 2-2 K.FFTLTAYAVPWTQK.E 727.6 1 19/26

215 acetyl-CoA acetyltransferase, mitochondrial precursor 45199.3 4557237

3 (3 0 0

0 0)

6178, 1 R.QAVLGAGLPISTPCTTINK.V 797.0 1 21/36

8478, 2-2 R.TPIGSFLGSLSLLPATK.L 819.3 1 17/32

8840, 1 R.TPIGSFLGSLSLLPATK.L 1249.1 1 21/32

216 surfeit locus protein 4 30393.8 19557691

5 (5 0 0

0 0)

8039, 2-2 R.LCLISTFLEDGIR.M 1757.8 1 21/24

8303, 1-2 R.LCLISTFLEDGIR.M 1024.5 1 16/24

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8322, 1-2 R.LCLISTFLEDGIR.M 1694.7 1 20/24

8411, 1 R.LCLISTFLEDGIR.M 1697.0 1 20/24

9398, 1-2 R.NLALGGGLLLLLAESR.S 1689.6 1 21/30

217 arginase-1 isoform 2 34734.7 10947139

2 (2 0 0

0 0)

7122, 1-2 K.TGLLSGLDIM*EVNPSLGK.T 800.0 1 17/34

7490, 1 K.RPIHLSFDVDGLDPSFTPATGTPVVGGLTYR.E

833.7 1 32/120

218 T-complex protein 1 subunit zeta isoform a 58023.9 4502643

2 (2 0 0

0 0)

5010, 1 R.AQAALAVNISAAR.G 551.2 1 19/24

10108, 1 K.VHAELADVLTEAVVDSILAIK.K 1387.3 1 22/40

219 aldo-keto reductase family 1 member C1 36788.1 5453543

2 (2 0 0

0 0)

6711, 1 K.DIVLVAYSALGSHR.E 806.8 1 16/26

8474, 1 K.NLQLDYVDLYLIHFPVSVKPGEEVIPK.D

856.1 1 30/104

220 sulfotransferase 1A2 34310.4 4507303

3 (3 0 0

0 0)

5489, 1 K.DVAVSYYHFYHMAK.V 563.0 1 19/26

7334, 2-2 K.THLPLALLPQTLLDQK.V 621.1 1 17/30

7583, 1-2 K.THLPLALLPQTLLDQK.V 862.4 1 20/30

221 argininosuccinate lyase isoform 1 51657.7 68303542

2 (2 0 0

0 0)

7293, 1 R.INVLPLGSGAIAGNPLGVDR.E 1516.2 1 24/38

9304, 1 R.QTCSTLSGLLWELIR.T 715.6 1 14/28

222 vigilin isoform b 137969.8

34519928

3

2 (2 0 0

0 0)

4546, 1-2 K.AACLESAQEPAGAWGNK.I 749.4 1 17/32

6794, 1 K.ASVITQVFHVPLEER.K 606.7 1 14/28

223 pyruvate kinase isozymes R/L isoform 1 61829.8 10835121

2 (2 0 0

0 0)

5975, 1-2 R.TGILQGGPESEVELVK.G 903.3 1 20/30

6630, 1-2 K.GVNLPGAQVDLPGLSEQDVR.D 550.2 1 18/38

224 epoxide hydrolase 2 62615.4 27597073

5 (5 0 0

0 0)

7002, 2-2 K.ASPSEVVFLDDIGANLKPAR.D 639.8 1 16/38

7232, 1-2 K.ASPSEVVFLDDIGANLKPAR.D 1537.3 1 22/38

7352, 1 K.ASPSEVVFLDDIGANLKPAR.D 1161.2 1 21/38

7512, 2-2 K.GFTTAILTNTWLDDR.A 1730.1 1 19/28

7763, 1-2 K.GFTTAILTNTWLDDR.A 1515.5 1 18/28

225 L-serine dehydratase/L-threonine deaminase 34625.1 33469958

2 (2 0 0

0 0)

7506, 1 K.LFQEHPIFSEVISDQEAVAAIEK.F 1550.4 1 33/88

7553, 1 K.ILVEPACGAALAAVYSHVIQK.L 777.6 1 19/40

226 heat shock protein HSP 90-alpha isoform 1 98160.4

15379259

0

4 (4 0 0

0 0)

5164, 1 R.ELISNSSDALDKIR.Y 789.6 1 17/26

5291, 2-2 R.NPDDITNEEYGEFYK.S 929.7 1 20/28

5442, 1-2 R.NPDDITNEEYGEFYK.S 524.2 1 20/28

5559, 1 R.NPDDITNEEYGEFYK.S 698.4 1 23/28

227 3-ketoacyl-CoA thiolase, peroxisomal isoform a 44291.8 4501853 2 (2 0 0

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0 0)

5739, 1 K.VNPLGGAVALGHPLGCTGAR.Q 1150.1 1 22/38

7170, 2-2 R.IAQFLSDIPETVPLSTVNR.Q 925.4 1 20/36

228 tubulin alpha-1B chain 50151.3 57013276

4 (4 0 0

0 0)

6938, 2-2 R.SIQFVDWCPTGFK.V 1497.3 1 19/24

8336, 2-2 R.LISQIVSSITASLR.F 748.2 1 14/26

8548, 1-2 R.LISQIVSSITASLR.F 1270.8 1 19/26

8619, 1 R.LISQIVSSITASLR.F 1275.1 1 19/26

229 60S ribosomal protein L18 21634.3 4506607

3 (3 0 0

0 0)

5025, 2-2 K.TAVVVGTITDDVR.V 1838.1 1 20/24

5184, 1-2 K.TAVVVGTITDDVR.V 1340.3 1 20/24

6696, 2-2 K.ILTFDQLALDSPK.G 1154.9 1 16/24

230 kynurenine 3-monooxygenase 55809.7

18796004

5

3 (3 0 0

0 0)

4606, 2-2 K.SQYILSVSR.E 1299.2 1 15/16

7062, 2-2 K.AVDSLEQISNLISR.- 2091.7 1 19/26

7277, 1-2 K.AVDSLEQISNLISR.- 2106.6 1 20/26

231 heat shock protein HSP 90-beta 83263.6 20149594

3 (3 0 0

0 0)

5358, 2-2 K.SLTNDWEDHLAVK.H 1287.2 1 17/24

5512, 1-2 K.SLTNDWEDHLAVK.H 1751.0 1 19/24

5529, 1-2 R.NPDDITQEEYGEFYK.S 910.2 1 17/28

232 short-chain dehydrogenase/reductase 3 33548.2 31543615

2 (2 0 0

0 0)

5721, 1-2 K.SQHINTLGQFWTTK.A 1615.9 1 19/26

6584, 1 R.VRFPNLFPPLKPETVAR.R 710.8 1 25/64

233 leukotriene-B(4) omega-hydroxylase 1 precursor 59853.2 13435391

3 (3 0 0

0 0)

4317, 2-2 R.SVINASAAIAPK.D 1492.7 1 17/22

6026, 2-2 R.VLTQLVATYPQGFK.V 959.5 1 16/26

6302, 1 R.VLTQLVATYPQGFK.V 1057.2 1 17/26

234 ras-related protein Rap-1b isoform 2 16033.3

35445935

0

4 (4 0 0

0 0)

7553, 2-2 K.SKINVNEIFYDLVR.Q 891.9 1 16/26

7914, 1 K.SKINVNEIFYDLVR.Q 1025.1 1 17/26

8430, 2-2 K.INVNEIFYDLVR.Q 1870.7 1 20/22

8709, 1-2 K.INVNEIFYDLVR.Q 1333.1 1 18/22

235 NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform 3 67523.9

31698315

8

2 (2 0 0

0 0)

7053, 1-2 K.VLFLLGADGGCITR.Q 1756.0 1 22/26

7524, 1-2 R.NDGAAILAAVSSIAQK.I 1364.9 1 21/30

236 importin-5 125544.3 24797086

2 (2 0 0

0 0)

3706, 2-2 K.TKENVNATENCISAVGK.I 1010.5 1 17/32

6462, 2-2 K.VIAALLQTMEDQGNQR.V 519.3 1 14/30

237 ATP synthase subunit O, mitochondrial precursor 23277.2 4502303

2 (2 0 0

0 0)

4913, 2-2 K.VAASVLNPYVK.R 1043.2 1 16/20

8320, 2-2 R.FSPLTTNLINLLAENGR.L 1449.9 1 19/32

238 acetolactate synthase-like protein 67867.3 21361361 2 (2 0 0

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0 0)

7084, 2-2 R.GALQAVDQLSLFRPLCK.F 525.3 1 13/32

8015, 1 R.FIFTLVGGHISPLLVACEK.L 1920.0 1 34/72

239 catenin beta-1 85496.2 4503131

3 (3 0 0

0 0)

7458, 1-2 R.NEGVATYAAAVLFR.M 1156.2 1 18/26

7576, 1 R.NEGVATYAAAVLFR.M 1293.9 1 18/26

10019, 1 R.GLNTIPLFVQLLYSPIENIQR.V 913.3 1 21/40

240 UDP-glucuronosyltransferase 1-1 precursor 59591.1 8850236

2 (2 0 0

0 0)

6562, 1 R.GHEIVVLAPDASLYIR.D 1065.2 1 20/30

10096, 1 R.EVTVQDLLSSASVWLFR.S 1030.2 1 20/32

241 oxysterol-binding protein 1 89420.0 4505531

2 (2 0 0

0 0)

5594, 1-2 R.LTEDLEYHELLDR.A 1374.8 1 19/24

5888, 1-2 R.SLSELESLKLPAESNEK.I 518.9 1 16/32

242 vesicle-trafficking protein SEC22b precursor 24593.2 94429050

2 (2 0 0

0 0)

5435, 1-2 R.NLGSINTELQDVQR.I 1004.7 1 19/26

7022, 1-2 R.IMVANIEEVLQR.G 2139.9 1 20/22

243 cytochrome P450 3A4 isoform 2 57255.8

32230235

1

3 (3 0 0

0 0)

5841, 2-2 K.GVVVMIPSYALHR.D 642.8 1 18/24

5996, 1-2 K.GVVVMIPSYALHR.D 648.3 1 18/24

6712, 1-2 K.LSLGGLLQPEKPVVLK.V 536.1 1 17/30

244 transmembrane 9 superfamily member 2 precursor 75775.3 4758874

2 (2 0 0

0 0)

5884, 2-2 K.RPSENLGQVLFGER.I 603.8 1 14/26

6638, 2-2 R.FCNPGFPIGCYITDK.G 969.3 1 22/28

245 OCIA domain-containing protein 1 isoform 1 27626.0 8923427

2 (2 0 0

0 0)

7974, 1-2 R.SVPLAATSMLITQGLISK.G 612.4 1 15/34

9071, 1 K.LILACIMGYFAGK.L 1881.2 1 19/24

246 carbonyl reductase [NADPH] 1 30374.7 4502599

2 (2 0 0

0 0)

7059, 1 R.SETITEEELVGLMNK.F 566.7 1 16/28

7776, 1 K.EYGGLDVLVNNAGIAFK.V 848.6 1 18/32

247 hydroxyacid oxidase 1 40924.1 11068137

2 (2 0 0

0 0)

5338, 1-2 R.NVAETDLSTSVLGQR.V 1833.5 1 22/28

8741, 1 K.GVQDVLEILKEEFR.L 1164.6 1 17/26

248 transmembrane protein 205 21197.7 63055043

3 (3 0 0

0 0)

3987, 2-2 R.GLGGEVPGSHQGPDPYR.Q 538.1 1 17/32

4064, 1-2 R.GLGGEVPGSHQGPDPYR.Q 507.1 1 17/32

6348, 2-2 R.TTAAMWALQTVEK.E 1570.6 1 18/24

249 apolipoprotein(a) precursor 226543.6

11629275

0

2 (2 0 0

0 0)

4368, 2-2 R.NPDAVAAPYCYTR.D 556.6 1 15/24

6263, 2-2 R.TPEYYPNAGLIMNYCR.N 639.3 1 19/30

250 sarcoplasmic/endoplasmic reticulum calcium ATPase 3 isoform a 109255.5 28373103

2 (2 0 0

0 0)

5806, 2-2 R.SLPSVETLGCTSVICSDK.T 574.6 1 17/34

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5842, 2-2 K.VGEATETALTCLVEK.M 1575.8 1 20/28

251 1-acylglycerol-3-phosphate O-acyltransferase ABHD5 39095.4 31542303

2 (2 0 0

0 0)

8516, 1 R.SGWLTGWLPTWCPTSISHLK.E 823.5 1 27/76

9058, 1 R.FDSDAEEVENQFVESIEEWR.C 811.1 1 19/38

252 7-alpha-hydroxycholest-4-en-3-one 12-alpha-hydroxylase 58068.1

16636272

4

2 (2 0 0

0 0)

7145, 1-2 K.LGALQHTPVLDSVVEETLR.L 503.0 1 23/72

10043, 1 K.EGISNWLGNMLQFLR.E 585.1 1 15/28

253 3-mercaptopyruvate sulfurtransferase isoform 2 33178.2 61835204

2 (2 0 0

0 0)

7654, 1 R.ALVSAQWVAEALR.A 2605.1 1 20/24

8176, 1-2 R.AGQPLQLLDASWYLPK.L 560.4 1 15/30

254 ER lumen protein retaining receptor 2 isoform 1 24421.7 5803050

2 (2 0 0

0 0)

8674, 1-2 R.ALYLVNWIWR.F 680.6 3 12/18

10124, 1 R.LTGDLSHLAAIVILLLK.I 992.4 1 16/32

255 MOSC domain-containing protein 2, mitochondrial precursor 38023.2 31542713

3 (3 0 0

0 0)

5289, 2-2 K.GVPVSEAECTAMGLR.S 707.0 1 18/28

5560, 2-2 R.CILTTVDPDTGVIDR.K 1289.3 1 20/28

5723, 1-2 R.CILTTVDPDTGVIDR.K 710.9 1 16/28

256 myosin-Ia 118399.8 4885503

3 (3 0 0

0 0)

5682, 1-2 K.LVMSYVAAVCGK.G 1444.1 1 16/22

7155, 2-2 K.EQLLQSNPVLEAFGNAK.T 967.0 1 17/32

7408, 1-2 K.EQLLQSNPVLEAFGNAK.T 879.9 1 17/32

257 60 kDa heat shock protein, mitochondrial 61054.2 31542947

2 (2 0 0

0 0)

4445, 2-2 K.NAGVEGSLIVEK.I 999.2 1 17/22

9665, 2-2 R.ALMLQGVDLLADAVAVTMGPK.G 1344.2 1 30/80

258 signal transducer and activator of transcription 3 isoform 1 88067.3 21618340

5 (5 0 0

0 0)

6027, 2-2 K.LLGPGVNYSGCQITWAK.F 550.1 1 15/32

6209, 1-2 K.LLGPGVNYSGCQITWAK.F 675.2 1 19/32

6294, 1 K.LLGPGVNYSGCQITWAK.F 511.2 1 17/32

6683, 2-2 R.GLSIEQLTTLAEK.L 1335.7 1 17/24

6897, 1-2 R.GLSIEQLTTLAEK.L 1698.8 1 19/24

259 plastin-2 70288.0

16761450

6

2 (2 0 0

0 0)

8711, 1 K.VNDDIIVNWVNETLR.E 1172.0 1 20/28

9114, 1 K.LNLAFIANLFNR.Y 1067.5 1 16/22

260 importin subunit beta-1 97169.7 19923142

3 (3 0 0

0 0)

7145, 2-2 K.LAATNALLNSLEFTK.A 1371.7 1 18/28

7510, 1 K.LAATNALLNSLEFTK.A 1909.1 1 22/28

8429, 1-2 R.AAVENLPTFLVELSR.V 542.2 1 18/28

261 dehydrogenase/reductase SDR family member 7 precursor 38298.5 7706318

2 (2 0 0

0 0)

6454, 2-2 K.EKDILVLPLDLTDTGSHEAATK.A 1125.5 1 30/84

7078, 2-2 K.DILVLPLDLTDTGSHEAATK.A 605.3 1 20/38

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262 vacuolar protein sorting-associated protein 13C isoform 1B 403082.1 66348091

2 (2 0 0

0 0)

6232, 1 K.LNAFCVIVCNEK.N 781.0 1 14/22

6934, 2-2 R.VLLFTDDVALVSK.A 629.4 1 14/24

263 cytochrome P450 2A7 isoform 1 precursor 56424.8 15147330

2 (2 0 0

0 0)

5153, 2-2 R.EALVDQAEEFSGR.G 1051.6 1 18/24

6890, 2-2 R.GEQATFDWVFK.G 1184.6 1 16/20

264 ras-related protein Rab-2B isoform 2 19005.1

25428134

5

3 (3 0 0

0 0)

5940, 2-2 K.LQIWDTAGQESFR.S 1690.7 1 19/24

6180, 1 K.LQIWDTAGQESFR.S 1007.0 1 19/24

6714, 2-2 R.GAAGALLVYDITR.R 1022.7 1 16/24

265 UDP-glucuronosyltransferase 2B10 isoform 2 precursor 50688.0

22121905

9

3 (3 0 0

0 0)

8162, 2-2 K.TEFENIIM*QLVK.R 585.9 1 13/22

8583, 2-2 K.TEFENIIMQLVK.R 1137.7 1 16/22

8858, 1-2 K.TEFENIIMQLVK.R 1379.9 1 16/22

266 S-adenosylmethionine synthase isoform type-1 43647.7 4557737

2 (2 0 0

0 0)

4740, 2-2 R.FVIGGPQGDAGVTGR.K 710.2 1 19/28

5180, 1 K.YLDEDTVYHLQPSGR.F 885.7 1 16/28

267 alpha-soluble NSF attachment protein 33232.5 47933379

2 (2 0 0

0 0)

4763, 1 K.LLEAHEEQNVDSYTESVK.E 644.2 1 17/34

7143, 2-2 K.NSQSFFSGLFGGSSK.I 1042.8 1 17/28

268 hypoxia up-regulated protein 1 precursor 111334.8 5453832

2 (2 0 0

0 0)

4725, 1 K.LYQPEYQEVSTEEQR.E 923.3 1 19/28

7390, 1-2 K.AANSLEAFIFETQDK.L 1063.1 1 17/28

269 protein disulfide-isomerase A6 precursor 48121.0 5031973

2 (2 0 0

0 0)

5483, 2-2 R.GSTAPVGGGAFPTIVER.E 626.8 3 16/32

7857, 1-2 R.TGEAIVDAALSALR.Q 1380.6 1 19/26

270 40S ribosomal protein S8 24205.0 4506743

3 (3 0 0

0 0)

5393, 2-2 K.ISSLLEEQFQQGK.L 1270.1 1 17/24

5531, 1-2 K.ISSLLEEQFQQGK.L 1358.2 1 17/24

5874, 1-2 R.IIDVVYNASNNELVR.T 984.5 1 17/28

271 methyltransferase-like protein 7B precursor 27774.6

16466380

5

3 (3 0 0

0 0)

4037, 2-2 R.VTCLDPNPHFEK.F 795.1 1 17/22

4121, 1-2 R.VTCLDPNPHFEK.F 780.3 1 17/22

8463, 2-2 K.SYFPYLMAVLTPK.S 734.9 1 16/24

272 receptor expression-enhancing protein 6 20733.0 19923919

3 (3 0 0

0 0)

7568, 2-2 K.CAFLLFCMAPR.P 1132.6 1 15/20

9197, 2-2 R.NLVTEVLGALEAK.T 1657.4 1 18/24

9338, 1 R.NLVTEVLGALEAK.T 1402.0 1 18/24

273 argininosuccinate synthase 46530.1 16950633

2 (2 0 0

0 0)

6173, 1-2 K.APNTPDILEIEFKK.G 555.4 1 16/26

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7632, 1 K.FAELVYTGFWHSPECEFVR.H 1001.1 1 19/36

274 TRAM adaptor with GOLD domain isoform 2 precursor 21232.8

25698510

2

2 (2 0 0

0 0)

4518, 1-2 K.SVIDYQTHFR.L 1038.1 1 14/18

6192, 2-2 K.FCFSNEFSTFTHK.T 744.3 1 14/24

275 erlin-2 isoform 1 37839.3 6005721

3 (3 0 0

0 0)

4185, 2-2 K.VAQVAEITYGQK.V 1922.8 1 18/22

4272, 1-2 K.VAQVAEITYGQK.V 2008.4 1 18/22

6215, 2-2 K.LSFGLEDEPLETATKEN.- 1439.2 1 20/32

276 Golgi apparatus protein 1 isoform 2 precursor 135994.8

22458681

5

3 (3 0 0

0 0)

5300, 2-2 K.AKDDSELEGQVISCLK.L 779.7 1 18/30

5352, 2-2 R.IIIQESALDYR.L 1430.5 1 15/20

5560, 1 K.AKDDSELEGQVISCLK.L 528.7 1 19/30

277 3-hydroxyacyl-CoA dehydratase 3 43159.3

11716824

8

3 (3 0 0

0 0)

4779, 2-2 R.LESEGSPETLTNLR.K 646.5 1 16/26

5876, 2-2 K.RPLFLAPDFDR.W 1251.1 1 18/20

6030, 1-2 K.RPLFLAPDFDR.W 712.8 1 15/20

278 protein disulfide-isomerase A4 precursor 72931.9 4758304

2 (2 0 0

0 0)

4301, 2-2 K.IDATSASVLASR.F 1688.5 1 18/22

6743, 1-2 K.YGIVDYMIEQSGPPSK.E 682.1 1 18/30

279 guanine nucleotide-binding protein G(I)/G(S)/G(T) subunit beta-2 37330.8 20357529

4 (4 0 0

0 0)

5967, 2-2 K.LIIWDSYTTNK.V 1103.4 1 16/20

6123, 1-2 K.LIIWDSYTTNK.V 784.4 1 14/20

6209, 1 K.LIIWDSYTTNK.V 1354.7 1 18/20

6288, 1 R.KACGDSTLTQITAGLDPVGR.I 510.2 1 16/38

280 myosin-14 isoform 3 232008.3

22483124

1

2 (2 0 0

0 0)

6172, 1 K.EQADFALEALAK.A 617.9 1 14/22

6777, 1-2 R.KQELELVVSELEAR.V 713.3 1 15/26

281 mitochondrial dicarboxylate carrier 31282.3 20149598

2 (2 0 0

0 0)

4722, 1-2 K.GEYQGVFHCAVETAK.L 890.3 1 17/28

5694, 1-2 R.GALVTVGQLSCYDQAK.Q 803.8 1 17/30

282 transmembrane emp24 domain-containing protein 9 precursor 27277.2 39725636

3 (3 0 0

0 0)

4500, 2-2 R.QLVEQVEQIQK.E 843.0 1 13/20

4637, 1-2 R.QLVEQVEQIQK.E 1134.4 1 17/20

7211, 1-2 K.FSLFAGGMLR.V 810.8 1 14/18

283 extended synaptotagmin-1 isoform 2 122855.6 14149680

2 (2 0 0

0 0)

4714, 1-2 K.LLAETVAPAVR.G 540.7 1 13/20

5286, 2-2 R.IHVLEAQDLIAK.D 1270.9 1 17/22

284 alpha-actinin-1 isoform b 103056.9 4501891

3 (3 0 0

0 0)

4958, 2-2 R.LAILGIHNEVSK.I 682.0 1 15/22

6018, 2-2 K.ICDQWDNLGALTQK.R 1629.4 1 18/26

6179, 1-2 K.ICDQWDNLGALTQK.R 623.0 1 16/26

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285 C4b-binding protein alpha chain precursor 67032.9 4502503

4 (4 0 0

0 0)

5142, 1-2 R.FSAICQGDGTWSPR.T 1430.0 1 19/26

6476, 2-2 K.LSLEIEQLELQR.D 1587.0 1 16/22

6674, 1-2 K.LSLEIEQLELQR.D 1934.2 1 17/22

6777, 1 K.LSLEIEQLELQR.D 1485.4 1 17/22

286 lysophospholipid acyltransferase 5 56034.7 42542394

3 (3 0 0

0 0)

4540, 2-2 K.LATSLGASEQALR.L 1382.6 1 19/24

6227, 2-2 K.VWLFETNPR.F 1164.4 1 15/16

6394, 1-2 K.VWLFETNPR.F 998.0 1 15/16

287 aspartyl/asparaginyl beta-hydroxylase isoform f 83267.4

25861394

3

2 (2 0 0

0 0)

6989, 1-2 K.GDWSQFTLWQQGR.R 681.5 1 13/24

8054, 1 R.LIFIVDVWHPELTPQQR.R 602.7 1 17/32

288 Golgi integral membrane protein 4 81879.8 7657138

2 (2 0 0

0 0)

4617, 2-2 K.SALAAAQTQVAEYK.Q 1504.3 1 18/26

4727, 2-2 K.FQSPYEEQLEQQR.L 602.1 1 13/24

289 vesicle-associated membrane protein-associated protein A isoform 1 32613.3 94721250

2 (2 0 0

0 0)

5735, 1-2 K.GPFTDVVTTNLK.L 1482.6 1 18/22

5842, 1-2 K.HEQILVLDPPTDLK.F 569.0 1 16/26

290 UDP-glucuronosyltransferase 1-6 isoform 1 precursor 60750.4 45827765

2 (2 0 0

0 0)

3874, 2-2 R.YQSFGNNHFAER.S 1239.3 1 17/22

4960, 2-2 R.SFLTAPQTEYR.N 625.1 1 15/20

291 ras-related protein Rab-2A isoform a 23545.4 4506365

2 (2 0 0

0 0)

5518, 1-2 K.TASNVEEAFINTAK.E 949.6 1 17/26

5794, 1-2 K.IQEGVFDINNEANGIK.I 516.0 1 15/30

292 ancient ubiquitous protein 1 45786.6 31712030

2 (2 0 0

0 0)

6075, 1 K.FPSSGPVTPQPTALTFAK.S 580.7 1 17/34

9089, 1 R.FSSWPFSIQDVVQPLTLQVQR.P 619.3 1 16/40

293 sarcoplasmic/endoplasmic reticulum calcium ATPase 2 isoform b 114756.1 24638454

2 (2 0 0

0 0)

3746, 2-2 R.NYLEPGKECVQPATK.S 650.5 1 18/28

7500, 2-2 K.DIVPGDIVEIAVGDKVPADIR.L 602.5 1 18/40

294 endoplasmic reticulum-Golgi intermediate compartment protein 1 32592.1 72534712

3 (3 0 0

0 0)

5098, 2-2 R.YDLSPITVK.Y 869.4 1 14/16

5207, 2-2 R.LTSNPLASHDYILK.I 985.9 1 17/26

5368, 1-2 R.LTSNPLASHDYILK.I 641.8 1 15/26

295 ubiquitin-like modifier-activating enzyme 1 117848.1 23510340

2 (2 0 0

0 0)

6664, 1 R.QFLFRPWDVTK.L 632.5 1 14/20

8873, 1 R.IYDDDFFQNLDGVANALDNVDAR.M 776.7 1 20/44

296 EH domain-containing protein 3 60886.7 7657056

2 (2 0 0

0 0)

5916, 1-2 R.KLFEAEEQDLFR.D 602.8 1 14/22

6314, 1 K.ELVNNLAEIYGR.I 729.6 1 14/22

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297 protein transport protein Sec31A isoform 1 133013.8

11625633

8

2 (2 0 0

0 0)

5225, 1-2 R.NPAVLSAASFDGR.I 1424.2 1 18/24

6724, 1-2 R.ISVYSIMGGSTDGLR.Q 801.8 1 17/28

298 probable ATP-dependent RNA helicase DDX17 isoform 1 80272.0 38201710

2 (2 0 0

0 0)

5662, 1-2 R.ELAQQVQQVADDYGK.C 609.4 1 16/28

5717, 1 K.FVINYDYPNSSEDYVHR.I 534.1 1 17/32

299 stromal interaction molecule 1 precursor 77422.9 21070997

2 (2 0 0

0 0)

4073, 1-2 K.ATGTSSGANSEESTAAEFCR.I 618.2 1 18/38

7918, 1-2 K.ALDTVLFGPPLLTR.H 708.8 1 16/26

300 beta-galactoside alpha-2,6-sialyltransferase 1 isoform a 46604.3 27765091

2 (2 0 0

0 0)

4570, 2-2 K.AKPEASFQVWNK.D 702.6 1 17/22

4691, 2-2 R.FNGAPTANFQQDVGTK.T 752.1 1 17/30

301 transferrin receptor protein 2 isoform 2 69617.1

33230917

1

2 (2 0 0

0 0)

6624, 2-2 R.GLTLQWVYSAR.G 1144.5 1 16/20

9303, 2-2 K.TSPLLTSLIESVLK.Q 744.9 1 17/26

302 alpha-tocopherol transfer protein-like 38514.9 85861250

2 (2 0 0

0 0)

3626, 1-2 K.AREELQEKPEWR.L 537.1 1 13/22

7307, 1 R.KEYPNLSTSLDDAFLLR.F 668.6 1 15/32

303 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform 1 precursor 70729.6 66346721

2 (2 0 0

0 0)

6975, 2-2 R.LGTPVLQALGDGDFVK.C 690.4 1 14/30

7959, 1 K.GVPLVYEAFNWR.H 769.6 1 18/22

304 L-xylulose reductase isoform 2 25742.7

30457197

5

4 (4 0 0

0 0)

4797, 2-2 R.TQADLDSLVR.E 1131.1 1 15/18

4919, 1-2 R.TQADLDSLVR.E 1278.9 1 16/18

5189, 2-2 R.AVIQVSQIVAR.G 1105.4 1 15/20

5340, 1-2 R.AVIQVSQIVAR.G 1216.1 1 16/20

305 clusterin preproprotein 52494.3

35559475

3

2 (2 0 0

0 0)

4410, 2-2 R.ELDESLQVAER.L 736.1 1 15/20

7444, 2-2 R.ASSIIDELFQDR.F 515.3 1 12/22

306 reticulon-4 isoform C 22395.3 5902016

2 (2 0 0

0 0)

6749, 1-2 K.DKVVDLLYWR.D 977.2 1 14/18

6832, 2-2 K.VVDLLYWR.D 696.5 1 12/14

307 60S ribosomal protein L7 29225.6 15431301

2 (2 0 0

0 0)

3327, 2-2 K.TTHFVEGGDAGNREDQINR.L 798.5 1 18/36

4577, 2-2 K.AGNFYVPAEPK.L 817.5 2 13/20

308 tubulin beta-8 chain isoform 1 49775.7 42558279

2 (1 1 0

0 0)

6194, 1-2 R.AVLVDLEPGTMDSVR.S 665.9 1 18/28

7094, 1-2 K.EAESCDCLQGFQLTHSLGGGTGSGMGTLLLSK.I

1051.2 1 31/124

309 cytochrome P450 4F8 precursor 59994.3 6005737 2 (1 1 0

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0 0)

5298, 1 R.LVHDFTDAVIQER.R 1465.7 1 19/24

7962, 2-2 K.TLDFIDVLLLSEDKNGK.E 615.8 2 14/32

310 cytochrome P450 4A22 59245.5 62952506

2 (1 1 0

0 0)

3974, 2-2 R.ACQLAHQHTDQVIQLR.K 997.7 1 25/60

5348, 1 R.NAFHENDTIYSLTSAGR.W 818.5 2 19/32

311 HLA class I histocompatibility antigen, Cw-1 alpha chain precursor 40648.3 52630342

2 (1 1 0

0 0)

5021, 2-2 R.SWTAADTAAQITQR.K 1643.7 1 20/26

6630, 1 R.AYLEGTCVEWLR.R 1615.3 1 19/22

312 guanine nucleotide-binding protein G(i) subunit alpha-2 isoform 1 40450.7 4504041

3 (1 2 0

0 0)

5268, 2-2 K.LLLLGAGESGK.S 1077.1 1 16/20

5392, 1 K.TTGIVETHFTFK.D 532.0 1 14/22

5398, 1-2 K.LLLLGAGESGK.S 933.4 1 16/20

313 myosin-11 isoform SM2A 223574.8 13124875

2 (1 1 0

0 0)

4038, 1-2 K.KEEELQAALAR.L 1122.4 1 16/20

5081, 1-2 K.KQELEEILHEM*EAR.L 674.9 2 13/26

314 heat shock 70 kDa protein 1A/1B 70051.8

19424807

2

3 (1 2 0

0 0)

4828, 2-2 K.AQIHDLVLVGGSTR.I 956.3 1 16/26

4961, 1-2 K.AQIHDLVLVGGSTR.I 706.7 1 16/26

6254, 1 R.ARFEELCSDLFR.S 795.1 1 16/22

315 electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial precursor 68495.0

11970374

6

2 (2 0 0

0 0)

8127, 2-2 K.SGILAAESIFNQLTSENLQSK.T 1596.8 1 23/40

8391, 1-2 K.SGILAAESIFNQLTSENLQSK.T 1121.5 1 20/40

316 short/branched chain specific acyl-CoA dehydrogenase, mitochondrial precursor 47485.1 4501859

1 (1 0 0

0 0)

8132, 1 R.LFDFQGLQHQVAHVATQLEAAR.L 1084.2 1 30/84

317 sepiapterin reductase 28048.3 4507185

2 (2 0 0

0 0)

8798, 1-2 R.VPADLGAEAGLQQLLGALR.E 1193.7 1 21/36

8808, 1-2 R.VPADLGAEAGLQQLLGALR.E 1583.9 1 22/36

318 erythrocyte band 7 integral membrane protein isoform a 31730.6 38016911

3 (3 0 0

0 0)

5164, 2-2 R.VQNATLAVANITNADSATR.L 1131.5 1 21/36

5451, 1 R.VQNATLAVANITNADSATR.L 770.3 1 20/36

5465, 1 R.VQNATLAVANITNADSATR.L 1177.9 1 21/36

319 phenylalanine-4-hydroxylase 51861.8 4557819

1 (1 0 0

0 0)

8457, 1 K.ILADSINSEIGILCSALQK.I 2088.2 1 34/72

320 retinal dehydrogenase 1 54861.5 21361176

2 (2 0 0

0 0)

6839, 1-2 K.LYSNAYLNDLAGCIK.T 778.5 1 17/28

6936, 1 K.LYSNAYLNDLAGCIK.T 2608.8 1 24/28

321 membrane primary amine oxidase 84621.4 4502119

1 (1 0 0

0 0)

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7678, 1 R.GDQDAGACEVNPLACLPQAAACAPDLPAFSHGGFSHN.-

599.3 1 30/144

322 glutathione S-transferase kappa 1 isoform a 25496.7 7705704

2 (2 0 0

0 0)

6105, 1-2 R.NEDITEPQSILAAAEK.A 916.9 1 21/30

6201, 1 R.NEDITEPQSILAAAEK.A 693.7 1 18/30

323 nicotinamide N-methyltransferase 29573.8 5453790

1 (1 0 0

0 0)

5918, 1-2 K.EIVVTDYSDQNLQELEK.W 1884.1 1 23/32

324 apolipoprotein C-III precursor 10852.2 4557323

1 (1 0 0

0 0)

4358, 2-2 K.DALSSVQESQVAQQAR.G 2145.4 1 22/30

325 inter-alpha-trypsin inhibitor heavy chain H1 isoform a 101388.4

15611962

5

1 (1 0 0

0 0)

5063, 1 R.FAHYVVTSQVVNTANEAR.E 1154.0 1 20/34

326 apoptosis-inducing factor 2 40526.4 14318424

1 (1 0 0

0 0)

6988, 1 K.TEYPEKEVTLIHSQVALADKELLPSVR.Q

972.0 1 30/104

327 annexin A2 isoform 2 38603.8

20986283

1

1 (1 0 0

0 0)

5697, 1 K.TDLEKDIISDTSGDFRK.L 2734.7 1 34/64

328 GDH/6PGL endoplasmic bifunctional protein precursor 88892.2 52145310

1 (1 0 0

0 0)

6966, 1 K.TAEDYQALNKDIEAQLQHAGLR.E 1056.1 1 31/84

329 cystathionine beta-synthase 60586.2 4557415

1 (1 0 0

0 0)

8319, 1 R.VQELGLSAPLTVLPTITCGHTIEILR.E

725.1 1 30/100

330 protein PRRC1 46700.9 18677735

1 (1 0 0

0 0)

7001, 2-2 R.GQDEASAGGIWGFIK.G 1676.4 1 21/28

331 rRNA 2'-O-methyltransferase fibrillarin 33784.0 12056465

1 (1 0 0

0 0)

7964, 1 K.VLYLGAASGTTVSHVSDIVGPDGLVYAVEFSHR.S

1076.8 1 32/128

332 POTE ankyrin domain family member F 121443.8

15379135

2

6 (6 0 0

0 0)

6024, 1 K.SYELPDGQVITIGNER.F 711.0 1 19/30

6200, 2-2 K.SYELPDGQVITIGNER.F 1029.6 1 20/30

6222, 2-2 K.SYELPDGQVITIGNER.F 1098.8 1 21/30

6366, 1-2 K.SYELPDGQVITIGNER.F 1046.0 1 21/30

6441, 1-2 K.SYELPDGQVITIGNER.F 932.6 1 20/30

12062, 1 K.SYELPDGQVITIGNER.F 585.0 1 16/30

333 plasminogen isoform 1 precursor 90568.6 4505881

1 (1 0 0

0 0)

5140, 2-2 R.NPDGDVGGPWCYTTNPR.K 1142.3 1 20/32

334 40S ribosomal protein S5 22876.3 13904870

1 (1 0 0

0 0)

6536, 2-2 R.VNQAIWLLCTGAR.E 1397.6 1 18/24

335 nicotinamide phosphoribosyltransferase precursor 55520.8 5031977

1 (1 0 0

0 0)

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8988, 1 R.TPAGNFVTLEEGKGDLEEYGQDLLHTVFK.N

901.9 1 28/112

336 cytochrome b-c1 complex subunit 1, mitochondrial precursor 52645.5 46593007

1 (1 0 0

0 0)

5106, 2-2 R.NALVSHLDGTTPVCEDIGR.S 1485.3 1 22/36

337 prolow-density lipoprotein receptor-related protein 1 precursor 504608.8

12601256

2

1 (1 0 0

0 0)

6988, 2-2 R.AALSGANVLTLIEK.D 1336.3 1 20/26

338 dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate

dehydrogenase complex, mitochondrial isoform 1 precursor

48755.0 19923748

1 (1 0 0

0 0)

7491, 1 K.ASAFALQEQPVVNAVIDDTTK.E 832.5 1 20/40

339 OCIA domain-containing protein 2 isoform 1 16953.4 62244044

1 (1 0 0

0 0)

8068, 1-2 K.VALAGLLGFGLGK.V 2056.5 1 21/24

340 coatomer subunit gamma-2 97621.8

10913434

9

3 (3 0 0

0 0)

8108, 2-2 R.SIATLAITTLLK.T 2042.7 1 19/22

8366, 1-2 R.SIATLAITTLLK.T 1916.1 1 19/22

8445, 1 R.SIATLAITTLLK.T 1859.0 1 19/22

341 large proline-rich protein BAG6 isoform a 119408.2

14915869

2

1 (1 0 0

0 0)

5060, 1 R.SFFHQHYLGGQEPTPSNIR.M 943.6 1 31/72

342 heterogeneous nuclear ribonucleoprotein U isoform a 90584.0 74136883

2 (2 0 0

0 0)

5331, 2-2 R.NFILDQTNVSAAAQR.R 2610.9 1 23/28

5591, 1 R.NFILDQTNVSAAAQR.R 1203.0 1 21/28

343 thymidine phosphorylase precursor 49955.2

16615892

2

1 (1 0 0

0 0)

8481, 1 R.ALPLALVLHELGAGR.S 1698.3 1 21/28

344 gamma-glutamyltransferase 5 isoform b 62260.8

15326688

5

1 (1 0 0

0 0)

7928, 1 R.ETVPASHAPSLLDQCAQALPLGTGAQWIGVPGELR.G

689.5 1 34/136

345 heterogeneous nuclear ribonucleoprotein H2 49263.3 9624998

2 (2 0 0

0 0)

6754, 2-2 R.STGEAFVQFASQEIAEK.A 1349.3 1 19/32

6971, 1-2 R.STGEAFVQFASQEIAEK.A 1085.9 1 18/32

346 fermitin family homolog 2 isoform 3 72396.9

20186182

3

1 (1 0 0

0 0)

5302, 1-2 R.ILEAHQNVAQMSLIEAK.M 1795.7 1 22/32

347 UDP-glucose 6-dehydrogenase isoform 1 55023.8 4507813

1 (1 0 0

0 0)

6886, 1 R.AVQALCAVYEHWVPR.E 1535.4 1 19/28

348 UDP-N-acetylglucosamine transporter 35984.2 6912668

1 (1 0 0

0 0)

4847, 2-2 R.VLHDEILNKPMETLK.L 1820.0 1 20/28

349 5'-AMP-activated protein kinase subunit gamma-1 isoform 1 37579.1 4506061

1 (1 0 0

0 0)

9923, 1 K.GIVSLSDILQALVLTGGEK.K 1579.8 1 20/36

350 transmembrane protein 33 27978.0 22458912 2 (2 0 0

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7 0 0)

6676, 2-2 R.ALLANALTSALR.L 2464.9 1 20/22

6875, 1-2 R.ALLANALTSALR.L 2337.2 1 20/22

351 gap junction beta-1 protein 32024.3 4504005

1 (1 0 0

0 0)

7572, 1 K.SSFICNTLQPGCNSVCYDQFFPISHVR.L

566.7 1 28/104

352 PRA1 family protein 3 21614.6 5453704

1 (1 0 0

0 0)

8559, 1 R.TPMGIVLDALEQQEEGINR.L 1073.4 1 20/36

353 B-cell receptor-associated protein 31 isoform b 27991.4

21351101

2

1 (1 0 0

0 0)

7638, 1 R.LVTLISQQATLLASNEAFK.K 1167.0 1 20/36

354 RNA-binding protein EWS isoform 2 68477.8 4885225

1 (1 0 0

0 0)

5794, 1 R.AGDWQCPNPGCGNQNFAWR.T 534.0 1 17/36

355 long-chain-fatty-acid--CoA ligase 4 isoform 2 79187.6 12669909

1 (1 0 0

0 0)

8548, 1 R.SVTHFDSLAVIDIPGADTLDKLFDHAVSK.F

1104.1 1 33/112

356 bile acid-CoA:amino acid N-acyltransferase 46298.9 4502351

1 (1 0 0

0 0)

9203, 1 R.KPEVTDLEYFEEAANFLLR.H 671.3 1 18/36

357 elongation factor 1-alpha 1 50140.5 4503471

3 (3 0 0

0 0)

4956, 2-2 K.YYVTIIDAPGHR.D 1105.9 1 17/22

5092, 1-2 K.YYVTIIDAPGHR.D 969.0 1 16/22

5096, 1-2 K.YYVTIIDAPGHR.D 1250.0 1 18/22

358 sec1 family domain-containing protein 1 isoform a 72379.4 33469966

1 (1 0 0

0 0)

6922, 2-2 K.ALTDAGCNLNPLQYIK.Q 747.8 1 16/30

359 ceramide synthase 2 44875.8 31077094

1 (1 0 0

0 0)

5543, 1-2 R.APPNATLEHFYLTSGK.Q 871.7 1 19/30

360 leucine-rich repeat-containing protein 59 34930.2 40254924

1 (1 0 0

0 0)

5993, 2-2 R.LVNLQHLDLLNNK.L 1216.0 1 15/24

361 serine/threonine-protein kinase PAK 2 58042.3 32483399

1 (1 0 0

0 0)

5112, 1 K.GTEAPAVVTEEEDDDEETAPPVIAPRPDHTK.S

845.8 1 36/120

362 cytochrome c oxidase subunit II 25564.8

25183111

0

1 (1 0 0

0 0)

6401, 1-2 R.ILYMTDEVNDPSLTIK.S 737.6 1 16/30

363 glucose-6-phosphate isomerase isoform 2 63146.7 18201905

1 (1 0 0

0 0)

9335, 1 K.ILLANFLAQTEALMR.G 1178.5 1 18/28

364 epiplakin 555623.1

20745273

5

1 (1 0 0

0 0)

5301, 1-2 R.LLEAQIATGGVIDPVHSHR.V 1021.3 1 31/72

365 proteasome activator complex subunit 1 isoform 1 28722.9 5453990

1 (1 0 0

0 0)

8612, 1 R.NAYAVLYDIILK.N 1593.4 1 18/22

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366 phosphatidate cytidylyltransferase 2 51417.7 20143480

1 (1 0 0

0 0)

5606, 1-2 R.AESAPLPVSADDTPEVLNR.A 695.9 1 19/36

367 retinol dehydrogenase 11 isoform 1 precursor 35386.0

16679526

8

1 (1 0 0

0 0)

4014, 2-2 K.EIQTTTGNQQVLVR.K 1493.7 1 19/26

368 L-lactate dehydrogenase A chain isoform 1 36688.5 5031857

1 (1 0 0

0 0)

9394, 1 K.LLIVSNPVDILTYVAWK.I 538.6 1 17/32

369 ATP-binding cassette sub-family D member 1 82936.5 7262393

1 (1 0 0

0 0)

4104, 2-2 R.GLQAPAGEPTQEASGVAAAK.A 850.0 1 22/38

370 ubiquitin-60S ribosomal protein L40 precursor 14728.2 77539055

1 (1 0 0

0 0)

5936, 1 K.TITLEVEPSDTIENVK.A 517.0 1 17/30

371 myeloperoxidase precursor 83868.1 4557759

1 (1 0 0

0 0)

8141, 1 R.VGPLLACIIGTQFR.K 1704.2 1 19/26

372 uridine 5'-monophosphate synthase 52221.3 4507835

2 (2 0 0

0 0)

7992, 1-2 R.AALGPLVTGLYDVQAFK.F 698.2 1 18/32

8153, 1 R.AALGPLVTGLYDVQAFK.F 683.5 1 17/32

373 voltage-dependent anion-selective channel protein 2 isoform 1 33371.4

29631733

7

1 (1 0 0

0 0)

5907, 2-2 K.YKWCEYGLTFTEK.W 727.7 1 13/24

374 annexin A5 35936.5 4502107

1 (1 0 0

0 0)

8600, 1 K.GLGTDEESILTLLTSR.S 1094.8 1 18/30

375 UDP-glucuronosyltransferase 2A3 precursor 60254.1

19321142

7

2 (2 0 0

0 0)

6921, 2-2 K.ANIIASALAQIPQK.V 560.1 1 16/26

7161, 1-2 K.ANIIASALAQIPQK.V 1619.8 1 21/26

376 clathrin interactor 1 isoform 1 70294.4

30707812

3

1 (1 0 0

0 0)

9280, 1-2 K.SLLLLAYLIR.N 1683.5 1 16/18

377 short-chain specific acyl-CoA dehydrogenase, mitochondrial precursor 44297.0 4557233

1 (1 0 0

0 0)

10034, 1 R.IGIASQALGIAQTALDCAVNYAENR.M

773.2 1 24/96

378 microsomal glutathione S-transferase 3 16516.2 4758714

3 (3 0 0

0 0)

7478, 2-2 R.IASGLGLAWIVGR.V 1957.6 1 20/24

7718, 1-2 R.IASGLGLAWIVGR.V 1999.4 1 20/24

7865, 1 R.IASGLGLAWIVGR.V 2303.8 1 21/24

379 dual specificity mitogen-activated protein kinase kinase 2 44423.9 13489054

1 (1 0 0

0 0)

5639, 2-2 K.ELEAIFGRPVVDGEEGEPHSISPR.P

730.2 1 26/92

380 peroxisomal membrane protein 2 22252.4 8923892

2 (2 0 0

0 0)

7938, 2-2 R.ALAQYLLFLR.L 1603.8 1 16/18

8204, 1-2 R.ALAQYLLFLR.L 1881.1 1 17/18

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381 COP9 signalosome complex subunit 7a 30276.4 7705330

1 (1 0 0

0 0)

5028, 1 K.VTTAAAAAATSQDPEQHLTELREPAPGTNQR.Q

577.5 1 31/120

382 protein odr-4 homolog isoform 1 51102.8 57527756

1 (1 0 0

0 0)

5129, 1 K.AKLDNLDEEWATEHACQVSR.M 721.2 1 25/76

383 inverted formin-2 isoform 1 135623.5

14999938

0

1 (1 0 0

0 0)

7683, 1 R.ALDELFEAIEQK.Q 651.8 1 17/22

384 fructose-1,6-bisphosphatase 1 36842.3

18908369

2

1 (1 0 0

0 0)

5813, 1 K.EAVLDVIPTDIHQR.A 800.9 1 19/26

385 lanosterol synthase isoform 1 83308.3 47933395

2 (2 0 0

0 0)

5991, 2-2 R.SVQLPDGGWGLHIEDK.S 1097.8 1 22/30

6243, 1 R.SVQLPDGGWGLHIEDK.S 1203.1 1 23/30

386 cytoplasmic aconitate hydratase 98398.2 8659555

1 (1 0 0

0 0)

7758, 1 K.QAPQTIHLPSGEILDVFDAAER.Y 1057.7 1 30/84

387 40S ribosomal protein S3a 29944.7 4506723

2 (2 0 0

0 0)

5902, 2-2 K.ACQSIYPLHDVFVR.K 1038.9 1 20/26

6057, 1-2 K.ACQSIYPLHDVFVR.K 892.1 1 19/26

388 alpha-enolase isoform 1 47168.7 4503571

1 (1 0 0

0 0)

7246, 1 R.HIADLAGNSEVILPVPAFNVINGGSHAGNK.L

1029.0 1 35/116

389 ras-related protein Rab-8A 23668.1 16933567

1 (1 0 0

0 0)

7377, 2-2 K.ANINVENAFFTLAR.D 1817.2 1 21/26

390 UDP-glucuronosyltransferase 1-9 precursor 59940.7 11276085

3 (3 0 0

0 0)

6237, 2-2 K.TYSTSYTLEDLDREFK.A 737.5 1 14/30

6425, 1-2 K.TYSTSYTLEDLDREFK.A 900.5 1 15/30

6500, 1 K.TYSTSYTLEDLDREFK.A 621.2 1 15/30

391 carbonic anhydrase 2 29245.8 4557395

1 (1 0 0

0 0)

8955, 1 K.AVQQPDGLAVLGIFLK.V 747.8 1 19/30

392 guanidinoacetate N-methyltransferase isoform a 26318.0 4503909

1 (1 0 0

0 0)

6633, 1-2 K.VQEAPIDEHWIIECNDGVFQR.L 537.7 1 25/80

393 cathepsin B preproprotein 37821.4 4503139

1 (1 0 0

0 0)

8638, 1 K.NGPVEGAFSVYSDFLLYK.S 1361.5 1 20/34

394 alcohol dehydrogenase 6 isoform 2 39072.4 4501939

1 (1 0 0

0 0)

7332, 1 K.LNLDPLITHTLNLDK.I 779.0 1 18/28

395 aldo-keto reductase family 1 member C4 37094.6

32565208

3

1 (1 0 0

0 0)

8950, 1 K.LWVDPNSPVLLEDPVLCALAK.K 702.0 1 18/40

396 60S ribosomal protein L27 15797.6 4506623

1 (1 0 0

0 0)

Page 112: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

5022, 1 K.NIDDGTSDRPYSHALVAGIDR.Y 506.2 1 23/80

397 60S ribosomal protein L19 23465.8 4506609

1 (1 0 0

0 0)

5436, 2-2 K.VWLDPNETNEIANANSR.Q 890.5 1 18/32

398 nodal modulator 1 precursor 134323.0 51944953

1 (1 0 0

0 0)

7433, 2-2 R.DGENYVVLLDSTLPR.S 1737.0 1 19/28

399 staphylococcal nuclease domain-containing protein 1 101996.3 77404397

1 (1 0 0

0 0)

4650, 1-2 R.SSHYDELLAAEAR.A 731.6 1 16/24

400 serine--pyruvate aminotransferase 43009.7 4557289

1 (1 0 0

0 0)

7998, 1-2 R.ESLALIAEQGLENSWR.Q 1053.3 1 18/30

401 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial

precursor 40750.3 4758768

1 (1 0 0

0 0)

8007, 2-2 R.LLQYSDALEHLLTTGQGVVLER.S 1103.8 1 28/84

402 malate dehydrogenase, cytoplasmic isoform 2 36425.9 5174539

2 (2 0 0

0 0)

4846, 1-2 K.VIVVGNPANTNCLTASK.S 563.2 1 16/32

5021, 1 K.VIVVGNPANTNCLTASK.S 590.0 1 18/32

403 40S ribosomal protein S3 26688.1 15718687

1 (1 0 0

0 0)

4401, 2-2 R.ELAEDGYSGVEVR.V 2064.0 1 20/24

404 scavenger receptor cysteine-rich type 1 protein M130 isoform a precursor 125450.2

34417911

0

2 (2 0 0

0 0)

6831, 1-2 K.EAEFGQGTGPIWLNEVK.C 869.6 1 16/32

6929, 1 K.EAEFGQGTGPIWLNEVK.C 766.0 1 16/32

405 neutral alpha-glucosidase AB isoform 3 precursor 109437.2 88900491

1 (1 0 0

0 0)

7707, 1 K.VLLVLELQGLQK.N 978.2 1 15/22

406 catechol O-methyltransferase isoform MB-COMT 30036.9 4502969

1 (1 0 0

0 0)

7700, 2-2 R.YLPDTLLLEECGLLR.K 1176.5 1 19/28

407 actin, gamma-enteric smooth muscle isoform 1 precursor 41876.7 4501889

1 (1 0 0

0 0)

5286, 1-2 R.VAPEEHPTLLTEAPLNPK.A 781.0 1 20/34

408 mitochondrial carnitine/acylcarnitine carrier protein 32943.5 4557403

1 (1 0 0

0 0)

4990, 1-2 R.ELIRDEGVTSLYK.G 1289.2 1 18/24

409 transmembrane emp24 domain-containing protein 10 precursor 24975.8 98986464

1 (1 0 0

0 0)

7767, 1-2 R.RLEDLSESIVNDFAYMK.K 1288.5 1 19/32

410 alpha-1-antitrypsin precursor 46736.2 50363217

1 (1 0 0

0 0)

9950, 1 K.ELDRDTVFALVNYIFFK.G 1066.6 1 17/32

411 probable ATP-dependent RNA helicase DDX5 69147.6 4758138

1 (1 0 0

0 0)

5188, 2-2 K.APILIATDVASR.G 1427.5 1 19/22

412 obg-like ATPase 1 isoform 1 44743.2 58761500

1 (1 0 0

0 0)

9036, 1 K.IPAFLNVVDIAGLVK.G 1360.6 1 18/28

413 melanoma inhibitory activity protein 3 precursor 213699.7 12289187 1 (1 0 0

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0 0 0)

6221, 2-2 R.TQTAISVVEEDLK.L 1131.8 1 17/24

414 mitochondrial import receptor subunit TOM70 67454.4 54607135

1 (1 0 0

0 0)

6698, 1-2 R.CAEGYALYAQALTDQQQFGK.A 556.1 1 16/38

415 malectin precursor 32233.6 7661948

2 (2 0 0

0 0)

6935, 2-2 K.VCALYIMAGTVDDVPK.L 982.2 1 17/30

7162, 1-2 K.VCALYIMAGTVDDVPK.L 1442.3 1 20/30

416 mevalonate kinase 42450.6 4557769

1 (1 0 0

0 0)

6191, 2-2 R.SPALQILLTNTK.V 1371.5 1 17/22

417 ras-related protein Rab-10 22540.8

25622201

9

3 (3 0 0

0 0)

8082, 2-2 K.AFLTLAEDILR.K 880.5 1 15/20

8333, 1-2 K.AFLTLAEDILR.K 1162.0 1 16/20

8444, 1 K.AFLTLAEDILR.K 1030.2 1 16/20

418 4-aminobutyrate aminotransferase, mitochondrial precursor 56438.6 38679946

1 (1 0 0

0 0)

9309, 1 K.NLLLAEVINIIK.R 1735.7 1 18/22

419 core histone macro-H2A.1 isoform 1 39183.2 20336746

1 (1 0 0

0 0)

7178, 1-2 R.HILLAVANDEELNQLLK.G 691.6 1 17/32

420 peroxisomal bifunctional enzyme isoform 1 79494.4 68989263

1 (1 0 0

0 0)

4760, 2-2 R.LHNALALIR.L 898.7 1 15/16

421 uncharacterized protein C8orf74 33768.7 91176323

2 (2 0 0

0 0)

7248, 2-2 R.QIQNTFAILDLK.L 878.9 1 14/22

7467, 1-2 R.QIQNTFAILDLK.L 1100.5 1 15/22

422 E3 ubiquitin-protein ligase MARCH5 31231.5 8923415

1 (1 0 0

0 0)

4385, 2-2 R.IPAEANPLADHVSATR.I 906.0 1 18/30

423 serum paraoxonase/arylesterase 2 isoform 1 39380.6 66529294

1 (1 0 0

0 0)

6154, 1-2 K.LFVYDPNNPPSSEVLR.I 588.3 1 18/30

424 E3 UFM1-protein ligase 1 89594.6 24308039

1 (1 0 0

0 0)

7677, 2-2 K.TYDLPGNFLTQALTQR.L 765.8 1 17/30

425 nuclear pore membrane glycoprotein 210 precursor 205108.8 27477134

1 (1 0 0

0 0)

5963, 1-2 K.AVDPSSVALVTLGSSK.E 911.3 1 19/30

426 alanine aminotransferase 1 54636.6 4885351

1 (1 0 0

0 0)

5134, 1 R.ALCVINPGNPTGQVQTR.E 701.8 1 18/32

427 eukaryotic translation initiation factor 3 subunit A 166568.5 4503509

1 (1 0 0

0 0)

7917, 1 R.FNVLQYVVPEVK.D 2219.1 1 20/22

428 heterogeneous nuclear ribonucleoprotein F 45671.6 4826760

1 (1 0 0

0 0)

7810, 2-2 K.ATENDIYNFFSPLNPVR.V 852.7 1 17/32

429 peroxisomal trans-2-enoyl-CoA reductase 32544.1 19923817

1 (1 0 0

0 0)

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6407, 1-2 K.ELLELGSNVVIASR.K 521.4 1 14/26

430 long-chain-fatty-acid--CoA ligase 6 isoform d 77670.3

32741232

3

2 (2 0 0

0 0)

5494, 2-2 R.VGFFQGDIR.L 1415.4 1 15/16

5624, 1-2 R.VGFFQGDIR.L 1350.6 1 15/16

431 multidrug resistance-associated protein 6 isoform 1 164872.8

19034302

3

1 (1 0 0

0 0)

4794, 2-2 K.GQVAESGSPAQLLAQK.G 961.7 1 17/30

432 60S ribosomal protein L3 isoform a 46108.6 4506649

1 (1 0 0

0 0)

4778, 1-2 K.AHLMEIQVNGGTVAEK.L 807.5 1 18/30

433 aflatoxin B1 aldehyde reductase member 3 37206.2 41152114

2 (2 0 0

0 0)

7937, 1-2 R.FYAFNPLAGGLLTGK.Y 1102.1 1 19/28

8060, 1 R.FYAFNPLAGGLLTGK.Y 1722.2 1 23/28

434 regulator of microtubule dynamics protein 2 isoform 2 47399.1

28304667

4

1 (1 0 0

0 0)

7672, 1-2 K.FCNLALLLPTVTK.E 963.6 1 15/24

435 annexin A1 38714.0 4502101

1 (1 0 0

0 0)

8370, 1 K.GLGTDEDTLIEILASR.T 610.5 1 16/30

436 beta-2-glycoprotein 1 precursor 38298.0

15326684

1

2 (2 0 0

0 0)

8454, 2-2 K.FICPLTGLWPINTLK.C 704.4 1 16/28

8811, 1 K.FICPLTGLWPINTLK.C 543.1 1 19/28

437 heterogeneous nuclear ribonucleoprotein A3 39594.6 34740329

1 (1 0 0

0 0)

5562, 1 K.IFVGGIKEDTEEYNLR.D 959.9 1 19/30

438 60S ribosomal protein L8 28024.4 15431306

1 (1 0 0

0 0)

3927, 1-2 R.ASGNYATVISHNPETK.K 836.0 1 17/30

439 3 beta-hydroxysteroid dehydrogenase type 7 isoform b 21322.2

21856368

4

1 (1 0 0

0 0)

6792, 1 K.LVYLVTGGCGFLGEHVVR.M 683.8 1 19/34

440 transmembrane emp24 domain-containing protein 2 precursor 22761.1 5803149

1 (1 0 0

0 0)

7366, 1-2 K.IVMFTIDIGEAPK.G 1219.3 1 16/24

441 eukaryotic translation initiation factor 3 subunit L isoform 1 66726.6 7705433

1 (1 0 0

0 0)

5278, 1-2 K.VFSDEVQQQAQLSTIR.S 606.8 1 17/30

442 beta-soluble NSF attachment protein 33556.8 44917606

1 (1 0 0

0 0)

8136, 1-2 R.LDQWLTTMLLR.I 1297.5 1 16/20

443 vesicle-associated membrane protein-associated protein B/C isoform 1 27228.2 4759302

1 (1 0 0

0 0)

4938, 2-2 K.VEQVLSLEPQHELK.F 881.4 1 17/26

444 GTP-binding protein SAR1a 22366.6

21741636

9

2 (2 0 0

0 0)

6165, 2-2 K.LVFLGLDNAGK.T 1284.5 1 18/20

6321, 1-2 K.LVFLGLDNAGK.T 1244.1 1 18/20

445 26S proteasome non-ATPase regulatory subunit 5 56195.4 4826952

1 (1 0 0

0 0)

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5538, 2-2 K.VFTAIANQPWAQK.L 1405.6 1 18/24

446 vesicle transport protein GOT1B 15425.6 7705636

1 (1 0 0

0 0)

8048, 2-2 R.VPVLGSLLNLPGIR.S 1821.4 1 19/26

447 cadherin-1 preproprotein 97455.4 4757960

1 (1 0 0

0 0)

6699, 1-2 R.DTANWLEINPDTGAISTR.A 762.5 1 17/34

448 UDP-glucuronosyltransferase 2B11 precursor 61037.9 4507823

2 (2 0 0

0 0)

4192, 2-2 R.FDGNKPDALGLNTR.L 632.0 1 18/26

4288, 1-2 R.FDGNKPDALGLNTR.L 584.7 1 18/26

449 60S ribosomal protein L23 14865.4 4506605

1 (1 0 0

0 0)

6774, 2-2 R.ISLGLPVGAVINCADNTGAK.N 576.3 1 18/38

450 beta,beta-carotene 9',10'-oxygenase isoform a 65673.7 82617624

1 (1 0 0

0 0)

7236, 1 R.NYIIFIEQPLK.M 1473.4 2 16/20

451 3-ketoacyl-CoA thiolase, mitochondrial 41923.9

16761448

5

1 (1 0 0

0 0)

6131, 2-2 K.DFTATDLSEFAAK.A 1825.9 1 19/24

452 glycerol-3-phosphate acyltransferase 1, mitochondrial precursor 93794.2

19035853

9

2 (2 0 0

0 0)

7259, 2-2 R.LIANLAEHILFTASK.S 691.3 1 13/28

7488, 1-2 R.LIANLAEHILFTASK.S 558.9 1 13/28

453 histone H2B type 1-O 13906.0 16306566

2 (2 0 0

0 0)

4408, 2-2 R.KESYSIYVYK.V 764.9 1 14/18

4504, 1-2 R.KESYSIYVYK.V 1158.9 1 16/18

454 N-terminal kinase-like protein isoform A 89630.9

11543024

1

1 (1 0 0

0 0)

4916, 2-2 K.LESVSEDPTQLEEVEK.D 743.5 1 17/30

455 aminopeptidase N precursor 109538.8

15726630

0

1 (1 0 0

0 0)

5956, 1 R.AQIINDAFNLASAHK.V 1180.9 1 19/28

456 heat shock-related 70 kDa protein 2 70020.5 13676857

1 (1 0 0

0 0)

3796, 1-2 K.STAGDTHLGGEDFDNR.M 1164.2 1 19/30

457 protein MON2 homolog 190356.9

11432655

2

1 (1 0 0

0 0)

7050, 2-2 R.AWDVLLDHIQSAALSK.N 820.8 1 15/30

458 peripherin 53650.5 21264345

1 (1 0 0

0 0)

5248, 1-2 K.NLQEAEEWYK.S 970.9 1 14/18

459 mannosyl-oligosaccharide glucosidase isoform 1 91917.0

14999960

6

2 (2 0 0

0 0)

7511, 2-2 R.LGPLLDILADSR.H 1392.0 1 18/22

7758, 1-2 R.LGPLLDILADSR.H 1367.5 1 17/22

460 mitochondrial glutamate carrier 1 34470.0 13375983

2 (2 0 0

0 0)

5483, 1-2 R.GVNEDTYSGILDCAR.K 857.0 1 18/28

5603, 1 R.GVNEDTYSGILDCAR.K 909.9 1 18/28

461 ras-related protein Rab-18 22976.9 10880989

2 (2 0 0

0 0)

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7395, 2-2 K.LDNWLNELETYCTR.N 816.9 1 15/26

7661, 1-2 K.LDNWLNELETYCTR.N 1002.1 1 15/26

462 neutral cholesterol ester hydrolase 1 isoform c 31167.6

22642395

0

1 (1 0 0

0 0)

7859, 2-2 R.SAPLIADQAVLQLLPK.T 742.0 1 20/30

463 14-3-3 protein epsilon 29173.7 5803225

1 (1 0 0

0 0)

7452, 2-2 K.LICCDILDVLDK.H 886.5 1 16/22

464 ADP-ribosylation factor 6 20082.0 4502211

2 (2 0 0

0 0)

7053, 2-2 R.DAIILIFANK.Q 1437.9 1 15/18

7276, 1-2 R.DAIILIFANK.Q 1608.5 1 15/18

465 sodium-coupled neutral amino acid transporter 3 55772.8 5870893

1 (1 0 0

0 0)

9548, 1 K.SELPLVIQTFLNLEEK.T 763.3 1 16/30

466 F-actin-capping protein subunit beta isoform 2 31350.3

33086467

9

2 (2 0 0

0 0)

5344, 1-2 K.GCWDSIHVVEVQEK.S 657.7 1 19/26

5478, 1 K.GCWDSIHVVEVQEK.S 562.7 1 17/26

467 catenin delta-1 isoform 2A 98048.4

33268822

2

1 (1 0 0

0 0)

6736, 1 K.ALSAIADLLTNEHER.V 1033.7 1 19/28

468 tyrosine aminotransferase 50399.2 4507369

1 (1 0 0

0 0)

5015, 1-2 R.EEIASYYHCPEAPLEAK.D 562.7 1 14/32

469 ras-related protein Rab-1A isoform 1 22677.6 4758988

1 (1 0 0

0 0)

7854, 1-2 K.NATNVEQSFMTMAAEIK.K 833.7 1 18/32

470 poly(rC)-binding protein 1 37497.6

22235215

1

1 (1 0 0

0 0)

6686, 1-2 R.IITLTGPTNAIFK.A 800.1 1 17/24

471 clarin-3 25321.2 22748685

1 (1 0 0

0 0)

6942, 1-2 K.FAVLEILNNSSQK.T 988.9 1 17/24

472 peroxiredoxin-4 precursor 30539.7 5453549

1 (1 0 0

0 0)

5386, 1 K.DYGVYLEDSGHTLR.G 1120.0 1 18/26

473 transmembrane 9 superfamily member 1 isoform b precursor 55250.5 62460635

1 (1 0 0

0 0)

6204, 2-2 K.SLSLGEVLDGDR.M 1356.5 1 17/22

474 asialoglycoprotein receptor 2 isoform a 35191.1 4502253

1 (1 0 0

0 0)

5060, 2-2 K.ADHDALLFHLK.H 1251.0 1 16/20

475 spectrin beta chain, brain 2 271291.8 5902122

1 (1 0 0

0 0)

6412, 1 R.VQAVDAVAAELAAER.Y 1413.7 1 18/28

476 proteasome inhibitor PI31 subunit 29816.5

14561142

6

1 (1 0 0

0 0)

5801, 1 R.IVSGIITPIHEQWEK.A 504.3 1 16/28

477 rab GDP dissociation inhibitor beta isoform 1 50662.9 6598323

1 (1 0 0

0 0)

9516, 1 K.VPSTEAEALASSLMGLFEK.R 521.2 1 14/36

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478 enoyl-CoA hydratase domain-containing protein 1 isoform 1 32997.0

15769451

6

2 (1 1 0

0 0)

6360, 2-2 R.ELYLEEALQNER.D 536.4 3 13/22

6542, 1-2 R.ELYLEEALQNER.D 1079.0 2 15/22

479 glutathione S-transferase A3 25301.5 24430144

2 (2 0 0

0 0)

7203, 1-2 R.WLLAAAGVEFEEK.F 1174.4 1 19/24

7224, 1-2 R.WLLAAAGVEFEEK.F 1701.3 1 18/24

480 oligosaccharyltransferase complex subunit OSTC 16829.2 24308271

2 (2 0 0

0 0)

6888, 2-2 R.VPFLVLECPNLK.L 1137.2 1 17/22

7234, 1 R.VPFLVLECPNLK.L 1236.7 1 17/22

481 patatin-like phospholipase domain-containing protein 3 52865.1 17196626

1 (1 0 0

0 0)

9020, 1 R.LSILPWDESILDTLSPR.L 581.3 1 17/32

482 cutaneous T-cell lymphoma-associated antigen 9 87952.4

22458677

3

1 (1 0 0

0 0)

6762, 2-2 R.SNSELEDEILCLEK.D 1268.1 1 17/26

483 inositol 1,4,5-trisphosphate receptor type 2 308061.1 95147335

1 (1 0 0

0 0)

6543, 2-2 K.DVGHNIYILAHQLAR.H 925.8 1 16/28

484 26S protease regulatory subunit 10B 45796.7

19553939

5

2 (2 0 0

0 0)

7494, 2-2 K.ALQSVGQIVGEVLK.Q 782.9 1 15/26

7745, 1-2 K.ALQSVGQIVGEVLK.Q 565.5 1 15/26

485 protein LYRIC 63836.5

22355591

7

1 (1 0 0

0 0)

3954, 2-2 K.NKGDSHLNVQVSNFK.S 538.9 1 13/28

486 HCLS1-binding protein 3 42780.0 68800430

1 (1 0 0

0 0)

8273, 1 K.DAELAGSPELLEFLGTR.S 673.2 1 19/32

487 cytochrome P450 3A43 isoform 2 57669.5 16933533

2 (2 0 0

0 0)

5192, 2-2 K.LQEEIDAVLPNK.A 1339.6 1 16/22

5480, 1 K.LQEEIDAVLPNK.A 1638.6 1 18/22

488 ATP synthase subunit gamma, mitochondrial isoform H (heart) precursor 32880.7 4885079

1 (1 0 0

0 0)

6599, 2-2 R.IYGLGSLALYEK.A 1586.7 1 19/22

489 galectin-4 35940.9 5453712

1 (1 0 0

0 0)

4392, 2-2 K.VGSSGDIALHINPR.M 1220.3 1 19/26

490 ras-related protein Rab-2B isoform 1 24214.2 21361884

1 (1 0 0

0 0)

5856, 1-2 K.SCLLLQFTDKR.F 1446.0 1 17/20

491 dual specificity mitogen-activated protein kinase kinase 4 44287.5 4506889

1 (1 0 0

0 0)

7200, 1-2 K.LCDFGISGQLVDSIAK.T 681.7 1 16/30

492 PREDICTED: POTE ankyrin domain family member I isoform 2 121281.5 88953571

1 (1 0 0

0 0)

4656, 1-2 K.IWHHTFYNELR.V 1107.1 1 16/20

493 galactokinase 42272.0 4503895 1 (1 0 0

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0 0)

5894, 1-2 R.SLETSLVPLSDPK.L 608.7 1 15/24

494 60S acidic ribosomal protein P0 34273.3 16933546

1 (1 0 0

0 0)

7554, 1 R.VLALSVETDYTFPLAEK.V 825.7 1 18/32

495 fibrinogen alpha chain isoform alpha-E preproprotein 94972.5 4503689

1 (1 0 0

0 0)

6251, 1 K.GLIDEVNQDFTNR.I 1301.6 1 19/24

496 thromboxane-A synthase isoform 1 60649.2

19597289

8

1 (1 0 0

0 0)

5885, 2-2 K.QVLVENFSNFTNR.M 768.0 1 15/24

497 pyruvate carboxylase, mitochondrial precursor 129632.7

10604929

2

1 (1 0 0

0 0)

9225, 1 K.YSLQYYMGLAEELVR.A 1002.9 1 18/28

498 vacuolar protein sorting-associated protein VTA1 homolog 33879.0 21361741

1 (1 0 0

0 0)

6460, 1 K.YAGSALQYEDVSTAVQNLQK.A 925.5 1 18/38

499 apolipoprotein O-like precursor 29158.7

11681261

0

1 (1 0 0

0 0)

7562, 2-2 K.MGVITVSGLAGLVSAR.K 800.1 1 16/30

500 perilipin-5 50791.1

11629217

2

1 (1 0 0

0 0)

4502, 1-2 R.SSVWEQDQQNVVQR.V 1451.6 1 17/26

501 signal recognition particle receptor subunit beta 29702.0

28479526

6

1 (1 0 0

0 0)

4178, 1-2 R.SAAPSTLDSSSTAPAQLGK.K 911.1 1 21/36

502 heterogeneous nuclear ribonucleoprotein C-like 1 32142.2 61966711

1 (1 0 0

0 0)

6760, 1 R.VFIGNLNTLVVK.K 1120.0 1 17/22

503 plastin-3 isoform 1 70810.6 7549809

1 (1 0 0

0 0)

5212, 1-2 R.QFVTPADVVSGNPK.L 597.2 1 15/26

504 cAMP-dependent protein kinase catalytic subunit alpha isoform 1 40589.3 4506055

1 (1 0 0

0 0)

7950, 1 R.ILQAVNFPFLVK.L 1445.0 1 17/22

505 acyl-CoA dehydrogenase family member 11 87263.9 38505218

1 (1 0 0

0 0)

5254, 2-2 R.VPATNLILGEGR.G 962.3 1 16/22

506 dynamin-2 isoform 5 97976.6

29975839

4

1 (1 0 0

0 0)

5454, 1 K.VPVGDQPPDIEYQIK.D 682.7 1 19/28

507 ras GTPase-activating-like protein IQGAP1 189250.1 4506787

1 (1 0 0

0 0)

6746, 1-2 R.EQLWLANEGLITR.L 825.0 1 14/24

508 guanine nucleotide-binding protein subunit alpha-12 44278.9 42476111

2 (2 0 0

0 0)

5268, 2-2 K.ILLLGAGESGK.S 1077.1 1 16/20

5398, 1-2 K.ILLLGAGESGK.S 933.4 1 16/20

509 APOBEC1 complementation factor isoform 1 64256.0 20357572

1 (1 0 0

0 0)

6174, 1 K.VTEGVVDVIVYPSAADK.T 560.0 3 15/32

510 ribonuclease inhibitor 49973.3 42822872 1 (1 0 0

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0 0)

7518, 1-2 R.TLWIWECGITAK.G 564.1 1 12/22

511 vitamin K-dependent gamma-carboxylase isoform 1 87560.4 21361163

1 (1 0 0

0 0)

8714, 1 K.LLGFEWTDLSSWR.R 776.9 1 15/24

512 guanine nucleotide-binding protein G(i) subunit alpha-1 40360.8 33946324

1 (1 0 0

0 0)

8978, 1 K.AVVYSNTIQSIIAIIR.A 537.3 1 15/30

513 cytoskeleton-associated protein 4 66022.0 19920317

2 (2 0 0

0 0)

8267, 2-2 K.VQSLQATFGTFESILR.S 600.6 1 13/30

8531, 1-2 K.VQSLQATFGTFESILR.S 539.0 1 12/30

514 beta-actin-like protein 2 42002.9 63055057

1 (1 0 0

0 0)

6195, 1-2 R.DLTDYLMK.I 863.1 1 11/14

515 ranBP2-like and GRIP domain-containing protein 4 197286.6

21105943

1

1 (1 0 0

0 0)

6764, 1-2 K.SGQSALYDALFSSQSPK.D 564.4 1 15/32

516 DCC-interacting protein 13-alpha 79663.1 6912242

1 (1 0 0

0 0)

7466, 1-2 K.SETEDSILHQLFIVR.F 697.1 1 15/28

517 leukotriene-B(4) omega-hydroxylase 2 isoform a 59846.4

11922056

2

2 (2 0 0

0 0)

6808, 2-2 K.VVLGLTLLR.F 728.9 1 14/16

7026, 1-2 K.VVLGLTLLR.F 761.4 1 14/16

518 UDP-glucose:glycoprotein glucosyltransferase 1 precursor 177187.7 9910280

1 (1 0 0

0 0)

9010, 1 R.ILASPVELALVVMK.D 739.0 1 16/26

519 HLA class I histocompatibility antigen, A-1 alpha chain precursor 40840.5 24797067

1 (1 0 0

0 0)

6344, 2-2 R.FIAVGYVDDTQFVR.F 717.0 1 17/26

520 leukotriene-B(4) omega-hydroxylase 2 isoform b 59738.4

31283683

3

1 (1 0 0

0 0)

7774, 2-2 K.VWMGPIFPVIR.F 1309.5 1 16/20

521 lipopolysaccharide-responsive and beige-like anchor protein isoform 2 319105.2

14823359

6

1 (1 0 0

0 0)

7516, 1-2 K.NAGLAFIELVNEGR.L 849.5 1 12/26

522 succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor 31629.6

11538709

4

1 (1 0 0

0 0)

6159, 2-2 K.LQDPFSLYR.C 558.6 2 13/16

523 gamma-glutamyl hydrolase precursor 35964.1 4503987

1 (1 0 0

0 0)

8764, 1 K.TAFYLAEFFVNEAR.K 1096.5 1 19/26

524 AP-3 complex subunit beta-1 121319.4 32484979

1 (1 0 0

0 0)

7464, 1 K.LLTQYILNLGK.Y 874.0 1 14/20

525 coatomer subunit delta isoform 1 57210.1 11863154

1 (1 0 0

0 0)

7700, 1-2 K.NSNILEDLETLR.L 1059.6 1 17/22

526 lanosterol 14-alpha demethylase isoform 1 precursor 57278.0 4503243

1 (1 0 0

0 0)

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8776, 2-2 K.IDDILQTLLDATYK.D 736.5 1 15/26

527 DNA damage-binding protein 1 126967.0

14852901

4

1 (1 0 0

0 0)

6045, 1-2 R.ALYYLQIHPQELR.Q 599.1 1 15/24

528 EH domain-containing protein 1 60626.4 30240932

1 (1 0 0

0 0)

7653, 1-2 R.VYIGSFWSHPLLIPDNR.K 603.8 1 14/32

529 exocyst complex component 5 81852.4 5730037

1 (1 0 0

0 0)

6244, 1-2 K.LHLIAQELPFDR.F 703.6 1 15/22

530 tubulin alpha-1C chain 49895.0 14389309

1 (1 0 0

0 0)

6894, 1-2 R.AVCMLSNTTAVAEAWAR.L 644.3 2 15/32

531 complement factor B preproprotein 85532.4 67782358

1 (1 0 0

0 0)

5212, 1 R.FLCTGGVSPYADPNTCR.G 635.8 1 16/32

532 serotransferrin precursor 77049.5 4557871

1 (1 0 0

0 0)

3398, 1-2 R.KPVEEYANCHLAR.A 961.1 1 18/24

533 histone

H1.0 20862.8 4885371

1 (1 0 0

0 0)

6135, 2-2 K.YSDMIVAAIQAEK.N 745.6 2 15/24

534 torsin-1A-interacting protein 1 66248.0 39753957

1 (1 0 0

0 0)

6683, 1-2 R.SQPAILLLTAAR.D 855.2 1 16/22

535 solute carrier organic anion transporter family member 3A1 isoform 2 74372.1

22283157

3

1 (1 0 0

0 0)

6995, 1-2 K.PGGSSGGGRSGELQGDEAQR.N 918.0 1 18/38

536 sterol 26-hydroxylase, mitochondrial precursor 60234.4 4503211

1 (1 0 0

0 0)

5595, 2-2 K.VGLQFLQR.Q 996.8 1 13/14

537 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial

precursor 42509.3 6681764

2 (2 0 0

0 0)

8207, 1-2 R.VFEISPFEPWITR.D 828.2 1 15/24

8318, 1 R.VFEISPFEPWITR.D 995.2 1 17/24

538 mitochondrial import receptor subunit TOM40 homolog 37892.8 5174723

1 (1 0 0

0 0)

5670, 1 K.FVNWQVDGEYR.G 950.2 1 15/20

539 6-phosphofructokinase, liver type 85017.9 48762920

1 (1 0 0

0 0)

8660, 1 R.SEWGSLLEELVAEGK.I 1180.8 1 19/28

540 calcium/calmodulin-dependent 3',5'-cyclic nucleotide phosphodiesterase 1C

isoform 1 72207.9 4826894

1 (1 0 0

0 0)

5622, 1-2 K.TALQQPEAIEKPK.A 794.6 3 13/24

541 heterogeneous nuclear ribonucleoproteins A2/B1 isoform A2 36005.7 4504447

1 (1 0 0

0 0)

7671, 1 K.LFIGGLSFETTEESLR.N 1095.6 1 22/30

542 ras-related protein Rap-1A 20987.1 4506413

1 (1 0 0

0 0)

5310, 2-2 R.QWCNCAFLESSAK.S 517.2 1 12/24

543 metal transporter CNNM2 isoform 3 60487.1 40068051

1 (1 0 0

0 0)

Page 121: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7210, 1 K.MAGGQAAAALPTWKMAARR.S 754.5 1 18/36

544 ras-related protein Rab-15 23517.8 38371739

1 (1 0 0

0 0)

5244, 1-2 R.IQIWDTAGQER.Y 882.1 1 15/20

545 alpha-2-macroglobulin precursor 163290.5 66932947

1 (1 0 0

0 0)

6628, 1 R.IAQWQSFQLEGGLK.Q 992.5 1 15/26

546 apolipoprotein L2 37092.1 13562090

1 (1 0 0

0 0)

3953, 1-2 R.ALAEEVEQVHR.G 1633.9 1 16/20

547 fatty acid-binding protein, liver 14208.3 4557577

2 (2 0 0

0 0)

5963, 2-2 K.AIGLPEELIQK.G 541.6 1 16/20

6110, 1-2 K.AIGLPEELIQK.G 529.9 1 16/20

548 protein DJ-1 19890.9 31543380

1 (1 0 0

0 0)

4720, 1-2 R.DVVICPDASLEDAKK.E 630.1 1 15/28

549 dynamin-like 120 kDa protein, mitochondrial isoform 1 111629.9

22483124

3

1 (1 0 0

0 0)

7446, 2-2 K.AKNEILDEVISLSQVTPK.H 773.0 1 18/34

550 alkylglycerol monooxygenase 51499.6 51972212

1 (1 0 0

0 0)

6762, 1-2 R.LDDALTSISAGVLSR.L 723.9 2 14/28

551 transmembrane protein 109 precursor 26209.8 13129092

2 (1 1 0

0 0)

6191, 2-2 R.EAPVDVLTQIGR.S 894.8 2 15/22

6346, 1-2 R.EAPVDVLTQIGR.S 763.8 2 14/22

552 erlin-1 39170.9

15480048

7

2 (2 0 0

0 0)

3941, 2-2 R.SVQTTLQTDEVK.N 951.9 1 15/22

4022, 1-2 R.SVQTTLQTDEVK.N 937.5 1 15/22

553 asialoglycoprotein receptor 1 isoform b 29143.8

30838736

3

1 (1 0 0

0 0)

6464, 2-2 R.WVCETELDKASQEPPLL.- 553.1 1 17/32

554 alpha-1,3-mannosyl-glycoprotein 2-beta-N-acetylglucosaminyltransferase 50878.0

16785777

8

1 (1 0 0

0 0)

7768, 1 R.GLLQQIGDALSSQR.G 550.0 1 13/26

555 lysosome-associated membrane glycoprotein 1 precursor 44882.1

11238062

8

1 (1 0 0

0 0)

8393, 1-2 R.FFLQGIQLNTILPDAR.D 555.8 1 13/30

556 MAP kinase-activating death domain protein isoform g 175915.5 18860873

1 (1 0 0

0 0)

7811, 2-2 R.VYLYEGLLGR.D 954.1 1 13/18

557 calpain-2 catalytic subunit isoform 2 71475.3

22570310

0

1 (1 0 0

0 0)

4721, 1-2 K.KPPPNLFKIIQK.A 582.2 4 13/22

558 calreticulin precursor 48141.1 4757900

1 (1 0 0

0 0)

6876, 2-2 R.FYALSASFEPFSNK.G 822.2 1 14/26

559 15-hydroxyprostaglandin dehydrogenase [NAD+] isoform 1 28977.1 31542939

1 (1 0 0

0 0)

4562, 2-2 K.VALVTGAAQGIGR.A 1153.6 1 17/24

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560 insulin receptor-related protein precursor 143719.0 31657140

1 (1 0 0

0 0)

5132, 1 R.DLFPNLAVIRGTR.L 525.8 4 13/24

561 splicing factor 3B subunit 1 isoform 1 145829.1 54112117

1 (1 0 0

0 0)

8670, 1 K.AIGPHDVLATLLNNLK.V 984.1 1 17/30

562 thioredoxin-related transmembrane protein 1 precursor 31791.0

15110129

2

1 (1 0 0

0 0)

6958, 2-2 K.DFINFISDKEWK.S 530.0 4 12/22

563 ADP-ribosylation factor GTPase-activating protein 2 isoform 1 56720.0 31543983

1 (1 0 0

0 0)

7905, 1-2 R.STELDSNWNWFQLR.C 506.4 1 11/26

564 ATP-binding cassette sub-family F member 1 isoform a 95925.1 69354671

1 (1 0 0

0 0)

6544, 1 K.IGFFNQQYAEQLR.M 523.3 1 12/24

565 histone

H3.1 15404.0 4504281

1 (1 0 0

0 0)

4478, 2-2 R.EIAQDFKTDLR.F 759.8 1 15/20

566 splicing factor, proline- and glutamine-rich 76148.9 4826998

1 (1 0 0

0 0)

3864, 1-2 R.FATHAAALSVR.N 1187.3 1 17/20

567 probable G-protein coupled receptor 110 isoform 1 precursor 101363.9 61743940

1 (1 0 0

0 0)

5547, 1-2 K.QNSSDLSAKPK.F 1077.2 1 15/20

568 serine/threonine-protein kinase OSR1 58021.8 4826878

2 (2 0 0

0 0)

7100, 2-2 K.SGVLDESTIATILR.E 557.5 1 16/26

7318, 1-2 K.SGVLDESTIATILR.E 794.0 1 14/26

569 NADH-cytochrome b5 reductase 1 34094.6 49574502

1 (1 0 0

0 0)

8056, 2-2 K.LGMIAGGTGITPMLQLIR.A 511.4 1 14/34

570 40S ribosomal protein S2 31324.2 15055539

1 (1 0 0

0 0)

8187, 2-2 K.SLEEIYLFSLPIK.E 1419.2 1 17/24

571 hemoglobin subunit epsilon 16202.7 4885393

3 (3 0 0

0 0)

6782, 1-2 R.LLVVYPWTQR.F 1113.0 1 15/18

6902, 1 R.LLVVYPWTQR.F 1135.5 1 15/18

12029, 1 R.LLVVYPWTQR.F 737.0 1 13/18

572 zinc finger protein 175 81609.1 6005970

1 (1 0 0

0 0)

9164, 1 K.ECGKVFIQRSELLTHQK.T 811.3 1 17/32

573 proteasome activator complex subunit 2 27401.4 30410792

1 (1 0 0

0 0)

3843, 2-2 R.ALVHERDEAAYGELR.A 558.2 1 14/28

574 RPS10-NUDT3 protein 33140.6

32111708

4

1 (1 0 0

0 0)

7979, 2-2 R.IAIYELLFK.E 833.7 1 13/16

575 3-keto-steroid reductase 38205.9 7705421

2 (2 0 0

0 0)

8013, 2-2 K.ALFFGLFSR.K 720.2 1 12/16

8271, 1-2 K.ALFFGLFSR.K 731.0 1 12/16

576 rho-related GTP-binding protein RhoB precursor 22123.3 4757764 2 (2 0 0

Page 123: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

0 0)

6510, 2-2 K.QVELALWDTAGQEDYDR.L 522.7 1 13/32

6672, 1-2 K.QVELALWDTAGQEDYDR.L 693.0 1 16/32

577 atlastin-3 60541.6 45827806

1 (1 0 0

0 0)

3776, 2-2 K.IYQGEDLPHPK.S 1108.9 1 15/20

578 bax inhibitor 1 isoform 1 26537.6

14874620

9

1 (1 0 0

0 0)

5727, 2-2 R.KINFDALLK.F 725.3 1 12/16

579 type I iodothyronine deiodinase isoform a 28774.2 4557522

1 (1 0 0

0 0)

4806, 2-2 R.LQAAHLLLAR.S 1063.4 1 15/18

580 dual specificity mitogen-activated protein kinase kinase 1 43438.8 5579478

1 (1 0 0

0 0)

8200, 1 K.LPSGVFSLEFQDFVNK.C 527.7 1 17/30

581 signal recognition particle receptor subunit alpha isoform 1 69810.8 23308697

1 (1 0 0

0 0)

7805, 1-2 K.DAAGIAMEAIAFAR.N 759.1 1 14/26

582 serine/threonine-protein phosphatase PGAM5, mitochondrial isoform 1 32004.2

28160413

6

1 (1 0 0

0 0)

11042, 2-2 R.VALRTLGDTGFM*PPDKITR.S 675.1 1 15/36

583 acid ceramidase isoform b 46503.3

18901154

6

1 (1 0 0

0 0)

5128, 2-2 R.WYVVQTNYDR.W 723.3 1 13/18

584 signal recognition particle 68 kDa protein 70729.2 24497620

1 (1 0 0

0 0)

7798, 1 K.DLPDVQELITQVR.S 904.4 1 17/24

585 protocadherin-10 isoform 1 precursor 112935.2 14589916

1 (1 0 0

0 0)

5555, 1-2 R.KELDGLLTNTR.A 1332.4 1 16/20

586 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 12496.6 4503253

1 (1 0 0

0 0)

5445, 2-2 R.FLEEYLSSTPQR.L 705.7 1 15/22

587 prostaglandin reductase 1 isoform 2 32894.9

22605613

0

1 (1 0 0

0 0)

5550, 1 K.HFVGYPTNSDFELK.T 856.9 1 17/26

588 beta-glucuronidase precursor 74731.4

26883419

2

1 (1 0 0

0 0)

5897, 1 K.SLLEQYHLGLDQK.R 548.1 2 15/24

589 G patch domain-containing protein 3 59337.6

20527745

8

1 (1 0 0

0 0)

7080, 1-2 K.AENEAFTLADLK.Q 542.2 1 12/22

590 phosphoinositide 3-kinase adapter protein 1 90397.3 45505139

1 (1 0 0

0 0)

6546, 2-2 R.DEELPTLLHFAAK.Y 592.0 1 16/24

591 heat shock cognate 71 kDa protein isoform 2 53517.3 24234686

1 (1 0 0

0 0)

5894, 2-2 K.DAGTIAGLNVLR.I 838.4 3 15/22

592 brefeldin A-inhibited guanine nucleotide-exchange protein 2 202036.4

15041798

6

1 (1 0 0

0 0)

7026, 2-2 K.VGCNPNEDVAIFAVDSLR.Q 566.7 1 14/34

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593 AP-1 complex subunit gamma-1 isoform b 91351.0 71772942

1 (1 0 0

0 0)

7704, 1-2 K.ELLYFLDSCEPEFK.A 530.9 1 13/26

594 peroxiredoxin-1 22110.2 4505591

1 (1 0 0

0 0)

4876, 1-2 R.TIAQDYGVLK.A 594.3 1 15/18

595 5'-AMP-activated protein kinase subunit beta-2 30302.0 4885561

1 (1 0 0

0 0)

6101, 1-2 K.KSDFEVFDALK.L 1257.5 1 15/20

596 phosphorylase b kinase regulatory subunit beta isoform a 124883.5 4505783

1 (1 0 0

0 0)

8916, 1 R.FNPILDMLAALKK.G 640.4 1 15/24

597 nitric oxide synthase, brain isoform 3 125112.6

32363543

1

1 (1 0 0

0 0)

9963, 2-2 -.MGSIMHPSQHARRPEDVR.T 508.8 1 14/34

598 stomatin-like protein 2 38533.8 7305503

1 (1 0 0

0 0)

5121, 2-2 K.AEQINQAAGEASAVLAK.A 525.4 1 17/32

599 uncharacterized protein LOC728392 12843.6

24084932

9

1 (1 0 0

0 0)

6332, 2-2 R.AGGRAQGGRAGQPPK.A 808.8 2 16/28

600 mast cell carboxypeptidase A precursor 48669.6

22131674

9

1 (1 0 0

0 0)

7481, 2-2 K.PTCRETMLAVK.F 723.7 3 13/20

601 tubulin alpha-4A chain 49924.1 17921989

1 (1 0 0

0 0)

7428, 2-2 R.AVFVDLEPTVIDEIR.N 641.5 1 15/28

602 rho GTPase-activating protein 1 50435.4 4757766

1 (1 0 0

0 0)

6060, 1 K.NPEQEPIPIVLR.E 642.5 1 16/22

603 transcriptional adapter 2-alpha isoform a 51505.6 68509270

1 (1 0 0

0 0)

6602, 1-2 K.FIESHALEFELRR.E 760.2 1 14/24

604 heterogeneous nuclear ribonucleoprotein M isoform a 77515.2 14141152

1 (1 0 0

0 0)

5982, 1-2 K.LKEVFSMAGVVVR.A 1242.5 1 17/24

605 UPF0554 protein C2orf43 37318.3 11345458

1 (1 0 0

0 0)

7536, 2-2 R.AFLLFPTIER.M 536.7 2 13/18

606 AP-2 complex subunit alpha-2 isoform 1 104088.9

33882768

5

1 (1 0 0

0 0)

5312, 1-2 K.THIETVINALK.T 986.4 1 15/20

607 heat shock 70 kDa protein 1-like 70374.6

12425649

6

2 (2 0 0

0 0)

4828, 2-2 K.AKIHDIVLVGGSTR.I 956.3 1 16/26

4961, 1-2 K.AKIHDIVLVGGSTR.I 706.7 1 16/26

608 X-ray repair cross-complementing protein 5 82704.0 10863945

1 (1 0 0

0 0)

6534, 1-2 K.SQLDIIIHSLK.K 549.6 2 13/20

609 heat shock protein beta-1 22782.3 4504517

1 (1 0 0

0 0)

6561, 1 R.LFDQAFGLPR.L 1314.6 1 16/18

610 enoyl-CoA delta isomerase 2, mitochondrial isoform 1 40182.8 45643119 1 (1 0 0

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0 0)

7868, 1-2 R.WLSDECTNAVVNFLSR.K 574.8 1 15/30

611 ras-related protein Rap-1b isoform 4 15355.6

35445935

6

1 (1 0 0

0 0)

4215, 2-2 K.YDPTIEDSYR.K 526.5 1 15/18

612 prolargin precursor 43809.6 4506041

1 (1 0 0

0 0)

6892, 1 K.LENLLLLDLQHNR.L 712.6 1 13/24

613 glutathione S-transferase A5 25721.8 24308514

1 (1 0 0

0 0)

5235, 1-2 R.AILNYIASK.Y 771.2 1 15/16

614 WASH complex subunit strumpellin 134285.3

12095285

1

1 (1 0 0

0 0)

7268, 1-2 K.DQILTDSRYNPR.I 583.1 2 14/22

615 ADP/ATP translocase 1 33064.2 55749577

1 (1 0 0

0 0)

7610, 1 R.YFPTQALNFAFK.D 612.7 1 17/22

616 vacuolar protein sorting-associated protein 13A isoform C 355859.0 66346672

1 (1 0 0

0 0)

6953, 2-2 K.TATGLLYIIETQK.V 677.3 1 13/24

617 cytochrome c oxidase subunit 7A2, mitochondrial precursor 12843.9

26211822

7

1 (1 0 0

0 0)

5356, 2-2 K.GGVADALLYR.A 856.4 1 13/18

618 squalene monooxygenase 63922.8 62865635

1 (1 0 0

0 0)

7262, 1 K.ELHAPLTVVADGLFSK.F 565.3 1 17/30

619 60S ribosomal protein L30 12784.0 4506631

1 (1 0 0

0 0)

5679, 1 K.LVILANNCPALR.K 594.3 1 15/22

620 polypeptide N-acetylgalactosaminyltransferase 2 precursor 64732.4 4758412

1 (1 0 0

0 0)

7506, 2-2 K.GGFDWNLVFK.W 776.1 2 12/18

621 serpin H1 precursor 46440.3

33336085

1

1 (1 0 0

0 0)

6844, 1-2 R.LYGPSSVSFADDFVR.S 689.4 1 15/28

622 alpha-tocopherol transfer protein 31749.4 4507723

1 (1 0 0

0 0)

4161, 2-2 R.AECPEISADLHPR.S 638.7 1 14/24

623 translocon-associated protein subunit alpha precursor 32235.1

16940400

9

1 (1 0 0

0 0)

7808, 2-2 K.GTEDFIVESLDASFR.Y 739.7 1 17/28

624 mitochondrial ribonuclease P protein 3 precursor 67315.0 62988276

1 (1 0 0

0 0)

8243, 1 R.ESQLLLNVVSQLAK.R 723.3 1 14/26

625 bcl-2-like protein 13 52722.8 45243501

1 (1 0 0

0 0)

7274, 1-2 K.ALQMLLSQPVTYQAFR.E 630.3 1 14/30

626 transmembrane emp24 domain-containing protein 5 isoform 1 precursor 26004.7

28216581

4

1 (1 0 0

0 0)

7155, 1-2 K.LEDILESINSIK.S 690.5 3 13/22

627 probable N-acetyltransferase 8 25618.9

11719051

7

1 (1 0 0

0 0)

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6460, 2-2 R.QWVVGLLSR.G 801.8 1 13/16

628 serine/threonine-protein phosphatase 6 regulatory subunit 2 isoform 4 101006.2

33927604

4

1 (1 0 0

0 0)

4481, 1-2 R.GPVQTHISEVIR.G 718.7 1 14/22

629 protein kinase C alpha type 76763.6 4506067

1 (1 0 0

0 0)

3765, 1-2 K.AEVADEKLHVTVR.D 505.1 1 12/24

630 leucine-rich repeat-containing G-protein coupled receptor 6 isoform 2 99294.1 62912472

1 (1 0 0

0 0)

6497, 2-2 R.LQELGFHNNNIK.A 1149.7 1 15/22

631 translocation protein SEC63 homolog 87996.4 6005872

1 (1 0 0

0 0)

5056, 2-2 R.VLLLSHLAR.M 611.7 2 12/16

632 transient receptor potential cation channel subfamily M member 2 171224.5 4507689

1 (1 0 0

0 0)

7547, 2-2 -.M*EPSALRK.A 879.1 1 12/14

633 protein transport protein Sec24B isoform a 137416.6

11238221

2

5 (0 5 0

0 0)

7822, 2-2 R.TYINPFVSFIDQR.R 889.0 1 17/24

8073, 2-2 R.TYINPFVSFIDQR.R 1003.9 1 17/24

8075, 1-2 R.TYINPFVSFIDQR.R 866.1 1 17/24

8309, 1-2 R.TYINPFVSFIDQR.R 1082.9 1 17/24

8440, 1 R.TYINPFVSFIDQR.R 1443.2 1 21/24

634 L-lactate dehydrogenase A-like 6B 41942.7 15082234

1 (0 1 0

0 0)

9394, 1 K.LIIVSNPVDILTYVAWK.L 538.6 1 17/32

635 chromosome-associated kinesin KIF4A 139880.1

11668612

2

2 (1 1 0

0 0)

6677, 2-2 R.WENIATILEAKCALK.Y 518.3 2 13/28

7005, 1 R.WENIATILEAKCALK.Y 631.5 1 14/28

636 myosin-X 237344.3

15435497

9

1 (0 1 0

0 0)

7467, 1-2 K.EEVSSARASIIDK.W 783.0 5 15/24

637 tubulin beta-6 chain 49856.8 14210536

1 (0 1 0

0 0)

7450, 2-2 K.M*ASTFIGNSTAIQELFK.R 637.7 2 16/32

638 E3 ubiquitin-protein ligase TRIM23 isoform alpha 64066.3 4502197

2 (0 2 0

0 0)

7053, 2-2 R.DALLLIFANK.Q 1437.9 1 15/18

7276, 1-2 R.DALLLIFANK.Q 1608.5 1 15/18

639 acyl-CoA dehydrogenase family member 9, mitochondrial precursor 68760.0 21361497

1 (0 1 0

0 0)

9512, 1 K.VWITNGGLANIFTVFAK.T 1022.7 2 19/32

640 myosin-VI 148712.8 92859701

2 (0 2 0

0 0)

8430, 1-2 K.FKTQLNLLLDK.L 1514.3 4 15/20

8501, 1 K.FKTQLNLLLDK.L 1581.9 3 15/20

641 zinc finger CCHC domain-containing protein 3 43547.1 29648305

1 (0 1 0

0 0)

7487, 1-2 R.EKMGWAQVVKNLAEK.K 831.7 3 16/28

642 carbonyl reductase [NADPH] 3 30850.1 4502601

1 (0 1 0

0 0)

Page 127: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7776, 1 K.EYGGLNVLVNNAAVAFK.S 800.2 2 18/32

643 importin subunit alpha-3 57810.5 34485722

1 (0 1 0

0 0)

4546, 1-2 K.IEVLQQHENEDIYK.L 580.5 2 15/26

644 gamma-parvin 37485.0 11545879

1 (0 1 0

0 0)

4850, 2-2 -.MEPEFLYDLLQLPK.G 1129.7 1 17/26

645 N-acetyl-beta-glucosaminyl-glycoprotein 4-beta-N-acetylgalactosaminyltransferase

2 114974.2 71043500

1 (0 1 0

0 0)

5594, 1-2 R.DRWGGEDWELLDR.I 838.1 2 16/24

646 gamma-aminobutyric acid receptor subunit beta-2 isoform 1 precursor 59150.1 12548785

2 (0 2 0

0 0)

8430, 2-2 R.LDVNKIFYKDIK.Q 877.1 2 14/22

8709, 1-2 R.LDVNKIFYKDIK.Q 639.4 3 13/22

647 ras-related protein Rab-1B 22171.0 13569962

1 (0 1 0

0 0)

7854, 1-2 K.NATNVEQAFM*TMAAEIK.K 645.5 2 16/32

648 fibroblast growth factor 10 precursor 23435.8 4758360

1 (0 1 0

0 0)

6936, 1 K.LYGSKEFNNDCKLK.E 794.0 2 16/26

649 transmembrane emp24 domain-containing protein 4 precursor 25942.6 33457308

1 (0 1 0

0 0)

4637, 1-2 R.QLLDQVEQIQK.E 741.5 2 15/20

650 cadherin-4 isoform 2 96396.6

35664022

1

3 (1 1 0

1 0)

5822, 2-2 K.VENPIDLYIYVIDM*NDNR.P 575.4 2 16/34

5986, 1-2 K.VENPIDLYIYVIDM*NDNR.P 542.3 1 15/34

6051, 1 K.VENPIDLYIYVIDM*NDNR.P 619.2 1 16/34

651 ras-related protein Rab-33B 25717.4 13786129

1 (0 1 0

0 0)

5244, 1-2 K.IQLWDTAGQER.F 882.1 1 15/20

652 ephrin type-A receptor 2 precursor 108265.8 32967311

1 (0 1 0

0 0)

5336, 1-2 R.TASVSINQTEPPK.V 742.5 2 15/24

653 T-complex protein 1 subunit alpha isoform a 60343.2 57863257

1 (0 1 0

0 0)

6174, 1 R.YINENLIVNTDELGR.D 1005.4 1 18/28

654 Golgi-specific brefeldin A-resistance guanine nucleotide exchange factor 1 isoform

1 206443.7 4758416

1 (0 1 0

0 0)

6840, 2-2 R.ENYVWNVLLHR.G 736.1 1 14/20

655 exportin-7 123906.4

15444889

2

1 (0 1 0

0 0)

5622, 1-2 R.NYVLNYLATRPK.L 966.1 1 14/22

656 dynein heavy chain 14, axonemal isoform 1 518563.2

22355593

5

1 (0 1 0

0 0)

5504, 2-2 K.EPNLENEKNLLDK.H 545.7 2 14/24

657 ATP-dependent DNA helicase Q4 133076.5

28400530

9

1 (0 1 0

0 0)

6680, 1-2 R.LKANLKGTLQAGPALGR.R 582.1 2 16/32

658 leiomodin-2 61675.0

15037850

3

1 (0 1 0

0 0)

6976, 1-2 K.QPNSILKEIK.N 890.9 2 15/18

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659 cytochrome P450 2J2 57610.3 18491008

1 (0 1 0

0 0)

5669, 1-2 R.NFGLGKKSLEER.I 806.2 3 14/22

660 thiosulfate sulfurtransferase 33428.7 17402865

1 (0 1 0

0 0)

6094, 1 K.TYEQVLENLESKR.F 643.1 2 13/24

661 inositol 1,4,5-trisphosphate receptor type 1 isoform 1 308536.7

26995469

0

1 (0 1 0

0 0)

6543, 2-2 R.NVGHNIYILAHQLAR.H 577.9 2 13/28

662 structural maintenance of chromosomes protein 2 135655.0

11034742

0

1 (0 1 0

0 0)

5830, 1-2 K.EQNNDSQLKIK.E 1143.1 2 14/20

663 testis-expressed sequence 2 protein 125997.8 38679909

1 (0 1 0

0 0)

5613, 2-2 K.GSVEEIM*SQPKQK.E 513.1 3 14/24

664 cytochrome P450 4F22 61957.9

15813853

0

1 (0 1 0

0 0)

6808, 2-2 R.VVVALTLLR.F 520.2 3 13/16

665 desmoglein-3 preproprotein 107532.4

11996471

8

1 (0 1 0

0 0)

5615, 2-2 K.PCREGEDNSKR.N 591.0 4 13/20

666 poly [ADP-ribose] polymerase 14 202798.2

15481319

9

1 (0 1 0

0 0)

7658, 2-2 K.VTIRPAATLVNEGR.P 508.0 2 13/26

667 peroxisomal membrane protein PEX14 41236.3 4758896

1 (0 1 0

0 0)

6630, 1-2 K.ATTSTNWILESQNINELK.S 544.2 2 17/34

668 uncharacterized protein C12orf56 isoform 2 53561.4

15379121

8

1 (0 1 0

0 0)

7486, 1-2 K.FLFPFHHTKANNKK.V 539.5 5 12/26

669 A disintegrin and metalloproteinase with thrombospondin motifs 20 preproprotein 214718.3

12443055

7

1 (0 1 0

0 0)

5858, 2-2 K.RSSEALEPM*PFR.T 748.3 2 14/22

670 annexin A3 36375.0 4826643

1 (0 1 0

0 0)

5331, 2-2 K.M*LISILTERSNAQR.Q 685.2 2 14/26

671 outer dense fiber protein 2 isoform 1 103066.6

31075040

0

1 (0 1 0

0 0)

7481, 2-2 K.DEMNKEIEAAR.R 749.9 2 13/20

672 usherin isoform B 575559.4

21984226

6

1 (0 1 0

0 0)

5667, 1 K.DRTSPSAPSGMEPPK.L 529.3 5 15/28

673 6-phosphofructokinase type C isoform 2 85315.5

33419170

1

1 (0 1 0

0 0)

4809, 1-2 R.GKKFTTDDSICVLGISK.R 672.8 2 14/32

674 titin isoform N2-B 2992860.

0

29104522

3

1 (0 1 0

0 0)

4358, 2-2 K.DEKSLVEESQLPEGR.K 563.8 5 13/28

675 leukocyte immunoglobulin-like receptor subfamily B member 5 isoform 3

precursor 52968.7

12598759

3

1 (0 1 0

0 0)

7744, 1 K.PVTLWCQGPLETEEYR.L 593.4 2 15/30

676 zinc finger protein 644 isoform 2 11723.8 41152091

1 (0 1 0

0 0)

Page 129: Supplemental Table and Figure Legends Table S1. Clinical ......23 calcium-binding mitochondrial carrier protein Aralar2 isoform 2 74175.1 7657581 24 probable saccharopine dehydrogenase

7672, 1-2 -.M*LIRQNLALDCK.Q 650.5 2 13/22

677 myotilin isoform a 55394.8 5803106

1 (0 1 0

0 0)

6932, 1 R.VTLLIKDVNKK.D 960.9 2 15/20

678 isochorismatase domain-containing protein 1 32236.5

10347198

7

1 (0 1 0

0 0)

5915, 1-2 K.GLGSTVQEIDLTGVK.L 592.9 2 15/28

679 X-linked interleukin-1 receptor accessory protein-like 2 precursor 78669.4 11225607

1 (0 1 0

0 0)

5444, 1-2 K.LLSLIKWK.G 687.5 1 13/14

680 exportin-5 136310.3 22748937

1 (0 1 0

0 0)

6971, 1-2 K.M*AEPFTKALDM*LDAEK.S 1009.4 2 18/30

681 killer cell lectin-like receptor subfamily F member 1 26562.4 7705574

1 (0 1 0

0 0)

6215, 2-2 K.IFFIKGPAKENSCAAIK.E 672.3 2 15/32

682 nucleoprotein TPR 267289.5

11415514

2

1 (0 1 0

0 0)

7277, 1-2 R.ELQELQDSLNAER.E 781.4 2 14/24

683 uncharacterized protein KIAA2026 228084.7

14861283

8

1 (0 1 0

0 0)

6656, 2-2 K.DVLNENLQR.K 621.1 2 13/16

684 guanylate cyclase soluble subunit alpha-3 isoform A 77452.1 67763816

1 (0 1 0

0 0)

4228, 2-2 K.NIQESLPQRK.T 835.4 2 13/18

685 ATPase family AAA domain-containing protein 5 207568.0 26080431

1 (0 1 0

0 0)

5993, 2-2 K.NNEEIGM*LLENNK.G 760.9 4 14/24

686 charged multivesicular body protein 5 isoform 1 24570.6

18940915

0

1 (0 1 0

0 0)

5823, 1-2 K.NKDGVLVDEFGLPQIPAS.- 678.4 2 18/34

687 coiled-coil domain-containing protein 164 87133.6

21741637

4

1 (0 1 0

0 0)

6377, 2-2 R.QRIEKLENEVK.T 685.3 3 12/20

688 DNA repair protein RAD52 homolog 46168.4

10963779

8

1 (0 1 0

0 0)

4641, 1-2 K.EAVTDGLKRALR.S 844.2 2 17/22

689 BTB/POZ domain-containing protein 18 77931.0

22302943

6

1 (0 1 0

0 0)

6178, 1-2 R.QNADNLSGTLLLK.R 1194.2 1 16/24

690 protein furry homolog 338871.6

11760635

5

2 (0 1 1

0 0)

5728, 2-2 R.DMPLNIFVK.I 664.4 3 11/16

5879, 1-2 R.DMPLNIFVK.I 736.0 5 11/16

691 60S ribosomal protein L14 23431.7 78000181

1 (0 1 0

0 0)

7775, 1-2 K.M*RNRIIK.N 634.4 3 9/12

692 ectopic P granules protein 5 homolog 292477.1 93204865

1 (0 0 1

0 0)

7467, 1-2 R.VEM*NENALVELK.K 860.3 2 14/22

693 tubulin beta-3 chain isoform 1 50432.4 50592996

1 (0 0 1

0 0)

7450, 2-2 K.MSSTFIGNSTAIQELFK.R 573.7 3 15/32

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694 cytochrome P450 2F1 precursor 55501.0 19743565

1 (0 0 1

0 0)

6682, 2-2 K.EALVDQGEEFSGR.G 584.1 3 15/24

695 uncharacterized protein C14orf45 61986.5

16629520

4

1 (0 0 1

0 0)

4002, 1-2 K.QDQEKDNM*IEKLK.Q 1513.9 1 17/24

696 muscle-related coiled-coil protein 41898.9 66472922

1 (0 0 1

0 0)

7487, 1-2 R.EMENAIKSVQIDLLK.L 869.3 2 16/28

697 uncharacterized protein C1orf168 82069.5

16670691

7

1 (0 0 1

0 0)

7923, 2-2 R.AKFQNLDAPPLPGPIK.F 817.4 2 15/30

698 meprin A subunit alpha precursor 84418.5

15307026

2

1 (0 0 1

0 0)

6677, 2-2 K.MTGSPSDRLVVWVRR.D 511.9 3 13/28

699 ras-related protein Rab-6B 23461.5 96975097

1 (0 0 1

0 0)

5244, 1-2 R.LQLWDTAGQER.F 882.1 1 15/20

700 LIM domain-binding protein 1 isoform 3 42809.1 4504969

1 (0 0 1

0 0)

4850, 2-2 R.LCVILEPM*QELMSR.H 852.3 3 15/26

701 39S ribosomal protein L55, mitochondrial isoform a 15128.3 31563362

1 (0 0 1

0 0)

7065, 1 R.RMLAM*PIDLDTLSPEER.R 667.1 1 16/32

702 heterogeneous nuclear ribonucleoprotein L isoform a 64132.4 52632383

1 (0 0 1

0 0)

5594, 1-2 R.RSGAMVKMAAAGGGGGGGR.Y 602.6 3 17/36

703 geminin 23565.0 7705682

1 (0 0 1

0 0)

5081, 1-2 K.QEEIKENIKNSSVPR.R 546.7 5 13/28

704 ret finger protein-like 1 35490.7

14940813

0

1 (0 0 1

0 0)

5331, 2-2 R.SGCITQNRQDLAER.F 521.0 4 12/26

705 matrix metalloproteinase-19 isoform rasi-1 preproprotein 57356.6 4505213

1 (0 0 1

0 0)

5879, 1-2 K.TLKYLLLGR.W 855.4 2 11/16

706 ras-related protein Rab-8B 23584.0 7706563

1 (0 0 0

1 0)

5244, 1-2 K.LQIWDTAGQER.F 882.1 1 15/20

707 tubulin polyglutamylase TTLL5 143576.5 50658079

1 (0 0 0

1 0)

6862, 2-2 K.LGGSVLGLSMEEIKVLRR.V 642.8 2 17/34

708 disintegrin and metalloproteinase domain-containing protein 12 isoform 1

preproprotein 99541.7 73747885

1 (0 0 0

1 0)

4458, 1-2 R.LLFTNKKTTIEK.L 500.3 2 13/22

709 FERM, RhoGEF and pleckstrin domain-containing protein 2 119887.7 7662310

1 (0 0 0

1 0)

6672, 1 K.KLEAVYKEFELQK.V 553.4 2 13/24

710 orphan sodium- and chloride-dependent neurotransmitter transporter NTT5 82199.5 55749589

1 (0 0 0

1 0)

6682, 2-2 R.NAEILLKLINLGK.L 1354.2 1 17/24

711 60S ribosomal protein L5 34362.4 14591909 1 (0 0 0

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0 1)

7438, 1-2 K.VGLTNYAAAYCTGLLLAR.R 526.7 3 15/34

712 gamma-interferon-inducible protein 16 isoform 1 82587.7

33086475

3

1 (0 0 0

0 1)

7487, 1-2 R.M*VKSLLSNDLKLNLK.M 776.7 4 15/28

713 U5 small nuclear ribonucleoprotein 200 kDa helicase 244504.7 40217847

1 (0 0 0

0 1)

5132, 1 K.PFGSEEQLLPVEK.L 594.3 2 14/24

Table S6. Identification of proteins associated with LDs of human livers.

Gene Name GI Uniprot

protein DEFINITION Reference

lipid droplet

PLIN1 22371820

3 O60240 perilipin-1 12

PLIN2 34577059 Q99541 perilipin-2 14,17,18,21,23~26

PLIN3 31737352

4 O60664 perilipin-3 6,7,9,11,13~15,17~26,29,30

PLIN4 12293719

5 Q96Q06 perilipin-4 7,11,14~16,21~23,29,30

lipid metabolism

APOB 30066960

5 P04114 apolipoprotein B-100 precursor 5,13,19,25

APOA1 4557321 P02647 apolipoprotein A-I preproprotein 15,16,18,19,24

SLC27A2 22749961

9 O14975

very long-chain acyl-CoA synthetase isoform

1

FASN 41872631 P49327 fatty acid synthase 8,13,15

LSS 22417755

6 G5E9Q9 lanosterol synthase isoform 2

ACSL3 42794752 O95573 long-chain-fatty-acid--CoA ligase 3 14,19,21

ACSL1 40807491 P33121 long-chain-fatty-acid--CoA ligase 1

SCCPDH(CGI-49) 55770836 Q8NBX0 probable saccharopine dehydrogenase 14,19,21,23,24

ADH4 71565152 P08319 alcohol dehydrogenase 4

ADH1B 34577061 P00325 alcohol dehydrogenase 1B 5

RDH10 25282469 Q8IZV5 retinol dehydrogenase 10 19

HSD17B6 19743808 O14756 17-beta-hydroxysteroid dehydrogenase type

6

HSD17B13 21003211

0 Q7Z5P4

17-beta-hydroxysteroid dehydrogenase 13

isoform A 26

HSD17B13 21003211

2 Q7Z5P4

17-beta-hydroxysteroid dehydrogenase 13

isoform B 26

HSD17B4 31348281

0 P51659

peroxisomal multifunctional enzyme type 2

isoform 1 18

HSD11B1 5031765 P28845 corticosteroid 11-beta-dehydrogenase

isozyme 1

ADH1C 4501933 P00326 alcohol dehydrogenase 1C 5

CYP4F11 19308317

8 Q9HBI6 cytochrome P450 4F11 precursor 13,18,19,25

HSD17B2 4504503 P37059 estradiol 17-beta-dehydrogenase 2

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HSD17B12 7705855 Q53GQ0 estradiol 17-beta-dehydrogenase 12

CYP4A11 15893724

2 Q02928 cytochrome P450 4A11 13,18,19,25

CYP2D6 40805836 P10635 cytochrome P450 2D6 isoform 1

CYP2C19 4503219 P33261 cytochrome P450 2C19 precursor

CYP2C9 13699818 P11712 cytochrome P450 2C9 precursor

CYP2A7 15147328 P20853 cytochrome P450 2A7 isoform 2 precursor

CYP2E1 10834998 P05181 cytochrome P450 2E1 precursor

CYP2C8 31189330

9 P10632 cytochrome P450 2C8 isoform b 13,18,19,25

NSDHL 8393516 Q15738 sterol-4-alpha-carboxylate 3-dehydrogenase,

decarboxylating 17,19,23,25,26

CYB5R3 19379482

6 P00387 NADH-cytochrome b5 reductase 3 isoform 2 17,19,23,25

ACACB 13414206

2 O00763 acetyl-CoA carboxylase 2 precursor 13

MGLL 6005786 Q99685 monoglyceride lipase isoform 1 19,26

ALDH3A2 73466520 P51648 fatty aldehyde dehydrogenase isoform 1

ACSL5 42794756 Q9ULC5 long-chain-fatty-acid--CoA ligase 5 isoform a

ACADVL 76496475 P49748-1 very long-chain specific acyl-CoA

dehydrogenase, mitochondrial isoform 2

HSD17B11 14297672

9 Q8NBQ5 estradiol 17-beta-dehydrogenase 11 19

membrane traffic

ANXA6 71773329 E5RFF0 annexin A6 isoform 1 15

SCP2 19923233 P22307 non-specific lipid-transfer protein isoform 1

proprotein 11

RAB10 25622201

9 P61026 ras-related protein Rab-10 17,19,21,24

AP2B1 4557469 P63010 AP-2 complex subunit beta isoform b

RAB14 19923483 P61106 ras-related protein Rab-14 14,21

USO1 4505541 O60763 general vesicular transport factor p115

ABCD3 4506341 P28288 ATP-binding cassette sub-family D member

3 isoform a 15

COPB1 7705369 P53618 coatomer subunit beta

LMAN1 5031873 P49257 protein ERGIC-53 precursor 18

SEC23A 38202214 Q15436 protein transport protein Sec23A

COPG2 10913434

9 Q9UBF2 coatomer subunit gamma-2

COPA 14853685

3 P53621 coatomer subunit alpha isoform 2 9

EHHADH 26187853

9

Q08426-

2 peroxisomal bifunctional enzyme isoform 2

CLTC 4758012 Q00610 clathrin heavy chain 1 5

cell signal

GNB2L1 5174447 P63244 guanine nucleotide-binding protein subunit

beta-2-like 1

RAP1B 35445935

0 P61224 ras-related protein Rap-1b isoform 2 25

STAT3 21618340 P40763 signal transducer and activator of

transcription 3 isoform 1 24

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IQGAP2 11608933

7 Q13576 ras GTPase-activating-like protein IQGAP2

GNB2 20357529 P62879 guanine nucleotide-binding protein

G(I)/G(S)/G(T) subunit beta-2 15,18

NAI2 4504041 P04899 guanine nucleotide-binding protein G(i)

subunit alpha-2 isoform 1 15,18

PDCD6IP 22027538 Q8WUM

4

programmed cell death 6-interacting protein

isoform 1

Chaperone

HSP90B1 4507677 P14625 endoplasmin 17

HSPA5 16507237 P11021 78 kDa glucose-regulated protein 17,19,23,26

HSPA1A 19424807

2 P08107 heat shock 70 kDa protein 1A/1B 19

CANX 10716563

P27824 calnexin precursor 19,23,26

HSP90AA1 15379259

0 P07900 heat shock protein HSP 90-alpha isoform 1 17

endoplasmic reticulum

CES1 68508965 P23141 Liver carboxylesterase 1 5

SURF4 19557691 O15260 surfeit locus protein 4

SEC24B 11238221

2 O95487 protein transport protein Sec24B isoform a

DDOST 20070197 P39656 dolichyl-diphosphooligosaccharide--protein

glycosyltransferase 48 kDa subunit precursor 19,23

SEC24A 11617478

0 O95486 protein transport protein Sec24A isoform 1

RTN4 24431935 Q9NQC3 reticulon-4 isoform A 19

RPN2 35493916 P04844

dolichyl-diphosphooligosaccharide--protein

glycosyltransferase subunit 2 isoform 1

precursor

FMO5 22131667

2 P49326

dimethylaniline monooxygenase [N-oxide-

forming] 5 isoform 1

RRBP1 11061122

0 Q9P2E9 ribosome-binding protein 1

CES1 68508957 P23141 liver carboxylesterase 1 isoform c precursor 5

P4HB 20070125 P07237 protein disulfide-isomerase 19

POR 12713903

3 P16435 NADPH--cytochrome P450 reductase 2,3,14,19,29~31

SLC27A5 13325057 Q9Y2P5 bile acyl-CoA synthetase

Mitochondria

SLC25A13 7657581 Q9UJS0 calcium-binding mitochondrial carrier

protein Aralar2 isoform 2

GLUD2 31377775 P49448 glutamate dehydrogenase 2, mitochondrial

CPS1 16979091

5 P31327

carbamoyl-phosphate synthase ,

mitochondrial isoform a precursor

CPS1 17029579

7 P31327

carbamoyl-phosphate synthase [ammonia],

mitochondrial isoform c

PHB2 22130758

4 Q99623 prohibitin-2 isoform 1

HMGCS2 5031751 P54868 hydroxymethylglutaryl-CoA synthase,

mitochondrial isoform 1 precursor

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PHB 4505773 P35232 prohibitin 32

HADHB 4504327 P55084 trifunctional enzyme subunit beta,

mitochondrial precursor

MAOA 4557735 P21397 amine oxidase [flavin-containing] A

SDHA 15641600

3 P31040

succinate dehydrogenase [ubiquinone]

flavoprotein subunit, mitochondrial

BDH1 44680133 Q02338 D-beta-hydroxybutyrate dehydrogenase,

mitochondrial precursor

MAOB 38202207 P27338 amine oxidase [flavin-containing] B

ATP5F1 32189394 P24539 ATP synthase subunit beta, mitochondrial

ATP5A1 4757810 P25705 ATP synthase subunit alpha, mitochondrial

HADHA 20127408 P40939 trifunctional enzyme subunit alpha,

mitochondrial 18

other proteins

HIST1H2AD 10800130 P20671 histone H2A type 1-D 10,13

PGM1 21361621 P36871 phosphoglucomutase-1 isoform 1

DHRS1 20986290

1 Q96LJ7

dehydrogenase/reductase SDR family

member 1 19

HBB 4504351 P68871 hemoglobin subunit delta

MGST1 9945306 P10620 microsomal glutathione S-transferase 1

METTL7A 89145417 Q9H8H3 methyltransferase-like protein 7A 19

VDAC1 4507879 P21796 voltage-dependent anion-selective channel

protein 1 15,18

EPHX1 20986283

7 P07099 epoxide hydrolase 1 19

SULT1A2 4507303 P50226 sulfotransferase 1A2

EPHX2 27597073 P34913 epoxide hydrolase 2

C4BPA 4502503 P04003 C4b-binding protein alpha chain precursor

POTEF 15379135

2 A5A3E0 POTE ankyrin domain family member F

UGT2A1 33360924

5 Q9Y4X1 UDP-glucuronosyltransferase 2A2

UGT2B28 33303381

1 Q9BY64

UDP-glucuronosyltransferase 2B28 isoform

2

UGT1A4 6005930 P22310 UDP-glucuronosyltransferase 1-4

UGT2B7 19019438

9 P16662 UDP-glucuronosyltransferase 2B7

UGT1A9 11276085 O60656 UDP-glucuronosyltransferase 1-9

UGT2B15 11651729

9 P54855 UDP-glucuronosyltransferase 2B15

SORD 15662757

1 Q00796 sorbitol dehydrogenase 5

TPP1 5729770 O14773 tripeptidyl-peptidase 1 preproprotein

PON1 19923106 P27169 serum paraoxonase/arylesterase 1 precursor

PHGDH 23308577 O43175 D-3-phosphoglycerate dehydrogenase 15

SULT2A1 29540545 Q06520 bile salt sulfotransferase

MTHFD1 22213663

9 P11586 C-1-tetrahydrofolate synthase, cytoplasmic

DIAPH1 11939575

8 O60610 protein diaphanous homolog 1 isoform 1

ALDH8A1 30150069

8 Q9H2A2

aldehyde dehydrogenase family 8 member

A1 isoform 3

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ALB 4502027 P02768 serum albumin preproprotein 17

FTCD 11140815 O95954 formimidoyltransferase-cyclodeaminase

UGP2 48255966 Q16851 UTP--glucose-1-phosphate

uridylyltransferase isoform a

DAK 20149621 Q3LXA3

bifunctional ATP-dependent

dihydroxyacetone kinase/FAD-AMP lyase

(cyclizing)

FMO3 50541961 P31513 dimethylaniline monooxygenase [N-oxide-

forming] 3

protein translation, modification degradation

EEF2 4503483 P13639 elongation factor 2

EEF1A2 4503475 Q05639 elongation factor 1-alpha 2 15

GCN1L1 54607053 Q92616 translational activator GCN1 13

SEC14L2 7110715 O76054 SEC14-like protein 2 isoform 1

CAND1 21361794 Q86VP6 cullin-associated NEDD8-dissociated protein

1

Cytoskeleton

SPTBN1 11238225

0 Q01082 spectrin beta chain, brain 1 isoform 1

PLEC1 41322908 Q15149-1 plectin isoform 1e

SPTAN1 19459550

9 Q13813 spectrin alpha chain, brain isoform 1

TLN1 22302941

0 Q9Y490 talin-1 15

MYH9 12667788 P35579 myosin-9

MYO1B 44889481 O43795 myosin-Ib isoform 2

JUP 12056468 P14923 junction plakoglobin

LMNA 27436946 P02545 prelamin-A/C isoform 1

VIM 62414289 P08670 vimentin 14,15,20,21,25

TUBA1A 17986283 Q71U36 tubulin alpha-1A chain

TUBA1B 57013276 P68363 tubulin alpha-1B chain 19

TUBB2A 4507729 Q13885 tubulin beta-2A chain 19

TUBB4B 5174735 P68371 tubulin beta-2C chain

ACTB 4501885 P60709 actin, cytoplasmic 1 19

CDH4 35664022

1 P55283 cadherin-4 isoform 2

CTNNA1 55770844 P35221 catenin alpha-1

DSP 58530840 P15924 desmoplakin isoform I 1,3,4,27,28

VIL1 19439423

7 P09327 villin-1

ACTN4 12025678 O43707 alpha-actinin-4 19

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Table S7. A list of LD proteins up-regulated and down-regulated in human fatty livers identified by means

of 2D-LD-MS/MS.

Table S7. A list for up-and down-regulated Lipid droplet proteins in fatty livers as compared with control livers.

Protein names MW Accession Peptide

(Hits)

Up-regulated lipid droplet proteins

Band 1

1 long-chain-fatty-acid--CoA ligase 1 77942.9 40807491 31

2 trifunctional enzyme subunit alpha 82999.1 20127408 5

3 stress-70 protein, mitochondrial 73680.1 24234688 4

4 protein disulfide-isomerase A4 72931.9 4758304 3

5 very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 2

68058.1 76496475 2

Band 2

1 acetolactate synthase-like protein 67867.3 21361361 9

2 carnitine O-palmitoyltransferase 2, mitochondrial precursor 73776.6 4503023 9

3 ATP-binding cassette sub-family D member 3 isoform a 75475.5 4506341 4

4 liver carboxylesterase 1 isoform c 62392.5 68508957 4

5 perilipin-1 55990.0 223718203 3

6 long-chain-fatty-acid--CoA ligase 5 isoform a 82262.5 42794756 3

7 bile acyl-CoA synthetase 75384.7 13325057 3

8 acyl-coenzyme A synthetase ACSM2A, mitochondrial 64223.9 58082049 3

9 eukaryotic translation initiation factor 3 subunit L isoform 1 66726.6 7705433 2

Band 3

1 ATP synthase subunit alpha, mitochondrial 59750.3 4757810 19

2 cytochrome P450 2E1 56848.6 10834998 13

3 glutamate dehydrogenase 2, mitochondrial 61433.6 31377775 7

4 UDP-glucuronosyltransferase 2B7 60720.3 190194389 6

5 aldehyde dehydrogenase, mitochondrial 56380.9 25777732 5

6 perilipin-2 48075.1 34577059 6

7 cytochrome P450 2C19 55944.8 4503219 6

8 glutamate dehydrogenase 1, mitochondrial 61397.5 4885281 5

9 UTP--glucose-1-phosphate uridylyltransferase isoform a 56939.9 48255966 5

10 cytochrome P450 1A2 58407.1 73915100 5

11 UDP-glucuronosyltransferase 2B15 61036.0 116517299 4

12 cytochrome P450 4A11 59347.5 158937242 3

13 dimethylaniline monooxygenase [N-oxide-forming] 3 60033.2 50541961 4

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14 cytochrome P450 2C18 55710.4 13699816 3

15 cytochrome P450 4A22 59245.5 62952506 4

16 cytochrome P450 2C9 55627.6 13699818 2

Band 4

1 epoxide hydrolase 1 52948.5 209862837 33

2 perilipin-3 isoform 1 47074.7 255958282 14

3 arylacetamide deacetylase 45733.4 68299767 8

4 eukaryotic initiation factor 4A-II 46402.0 83700235 4

5 sulfide:quinone oxidoreductase, mitochondrial 49960.4 10864011 4

6 annexin A7 isoform 1 50315.4 4502111 4

7 synaptic vesicle membrane protein VAT-1 homolog 41920.0 18379349 4

8 argininosuccinate synthase 46530.1 16950633 3

9 adipocyte plasma membrane-associated protein 46480.1 24308201 2

Band 5

1 alcohol dehydrogenase 4 40221.5 71565152 3

Band6

1 17-beta-hydroxysteroid dehydrogenase 13 isoform B 29604.7 210032112 50

2 estradiol 17-beta-dehydrogenase 11 32935.6 142976729 13

3 retinol dehydrogenase 16 35673.2 150247226 8

4 NADH-cytochrome b5 reductase 3 isoform 2 31628.5 193794826 6

5 annexin A4 36084.8 4502105 6

6 electron transfer flavoprotein subunit alpha, mitochondrial isoform a

35079.3 4503607 5

7 2,4-dienoyl-CoA reductase, mitochondrial 36067.5 4503301 4

8 17-beta-hydroxysteroid dehydrogenase 13 isoform A 33655.2 210032110 6

9 short-chain dehydrogenase/reductase 3 33548.2 31543615 3

10 monoglyceride lipase isoform 1 34292.3 6005786 3

11 40S ribosomal protein S3a 29944.7 4506723 3

Band 7

1 prohibitin 29803.9 4505773 6

2 17-beta-hydroxysteroid dehydrogenase type 6 35965.5 19743808 5

3 vesicle-associated membrane protein-associated protein B/C isoform 1

27228.2 4759302 2

Down-regulated lipid droplet proteins

Band 8

1 ferritin light chain 20019.5 20149498 8

2 ferritin heavy chain 21225.5 56682959 3

3 solute carrier family 2, facilitated glucose transporter member 2

57489.2 4557851 2

4 ubiquitin-60S ribosomal protein L40 precursor 14728.2 77539055 2

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5 palmitoyl-protein thioesterase 1 isoform 1 precursor 34193.3 4506031 2

Band 9

1 myosin-9 226529.8 12667788 13

2 talin-1 269764.4 223029410 3

3 putative ATP-dependent RNA helicase DHX33 isoform 1 78873.6 20336302 2

4 hemoglobin subunit beta 15998.3 4504349 2

Band10

1 heat shock protein HSP 90-alpha isoform 1 98160.4 153792590 3

2 heat shock protein HSP 90-beta 83263.6 20149594 5

3 mannosyl-oligosaccharide glucosidase isoform 1 91917.0 149999606 2

4 calnexin precursor 67567.8 10716563 2

Band 11

1 serum albumin preproprotein 69366.4 4502027 24

2 bile acyl-CoA synthetase precursor 75384.7 13325057 8

3 dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 precursor

68569.0 4506675 7

4 heat shock 70 kDa protein 1A/1B 70051.8 194248072 3

5 calcium-binding mitochondrial carrier protein Aralar2 isoform 2

74175.1 7657581 2

6 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform 1 precursor

70729.6 66346721 2

7 annexin A6 isoform 1 75872.8 71773329 2

8 calcium-binding mitochondrial carrier protein Aralar1 74761.4 21361103 2

Band12

1 prostaglandin reductase 1 isoform 2 32894.9 226056130 7

2 elongation factor 1-alpha 2 50469.8 4503475 4

3 glyceraldehyde-3-phosphate dehydrogenase 36053.0 7669492 4

4 60S ribosomal protein L4 47697.0 16579885 3

5 actin-related protein 3 47370.9 5031573 3

6 elongation factor 1-alpha 1 50140.5 4503471 3

7 glycerol-3-phosphate dehydrogenase [NAD+], cytoplasmic 37567.4 33695088 3

8 thioredoxin domain-containing protein 5 isoform 1 precursor 47628.6 42794771 2

9 aldo-keto reductase family 1 member C4 37094.6 325652083 3

10 methyltransferase-like protein 7A precursor 28318.9 89145417 3

11 annexin A9 38363.3 145864465 2

12 transaldolase 37539.9 5803187 2

13 aldo-keto reductase family 1 member C1 36788.1 5453543 2

14 vimentin 53651.3 62414289 2

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Table S8. 4 KEGG pathways enriched in 58 LD proteins up-regulated in fatty liver.

Category Term Genes PValue

KEGG_PATHWAY hsa00830:Retinol metabolism

190194389,

158937242,

73915100,

4503219,

71565152,

150247226,

31543615,

13699818,

116517299,

62952506,

13699816

8.22E-13

KEGG_PATHWAY hsa00980:Metabolism of xenobiotics

by cytochrome P450

190194389,

10834998,

73915100,

209862837,

4503219,

71565152,

13699818,

116517299,

13699816

3.32E-09

KEGG_PATHWAY hsa00982:Drug metabolism

50541961,

190194389,

10834998,

73915100,

4503219,

71565152,

13699818,

116517299,

13699816

4.35E-09

KEGG_PATHWAY hsa00591:Linoleic acid metabolism

10834998,

73915100,

4503219,

13699818,

13699816

3.05E-05

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Figure S1. Histological and morphological examination of hepatic tissues and LDs in human subjects

with or without simple steatosis. A) Light micrograph of representative normal liver (left) and non-

alcoholic fatty liver (right) stained with HE. B) Oil red O staining demonstrating massive neutral lipid

accumulation in fatty liver (right panel). Little staining was observed in normal liver (left panel). C)

Masson staining of normal liver and non-alcoholic fatty liver. Note: no fibrotic lesion was observed in

either normal or steatotic liver. D) Electron micrographs of sections of livers stained with 2% (w/v) uranyl

acetate. The arrowheads indicate the LDs, asterisk indicates the nuclei, magnification 40,000×.

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Figure S2. Identification of the LD-associated protein expression profile of human livers without

NAFLD. A) LD proteins isolated from 3 normal human livers were processed for gel electrophoresis and

stained with silver staining. Samples from 3 livers were processed for mass spectrometry. B) Venn diagram

shows the cross-correlation of identified LD proteins from 3 samples. Numbers inside the circle or overlaps

separately represent proteins only belonging to or common to corresponding data sets. Note: 148 LD

proteins were found to be shared by all 3 samples. C) All human liver LD proteins identified by 2D-LC-

MS/MS were categorized by subcellular distributions and known functions based on literature or NCBI

online sources.

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Figure S3. Bioinformatics analysis of identified LD proteins in fatty livers and control livers. A)

Association network of identified LD proteins in fatty livers and control livers by a website program

STRING against human database. Network edges represent predicted functional associations with different

line colors standing for various types of evidence used for predicting: red, fusion evidence; green,

neighborhood evidence; blue, co-occurrence evidence; purple, experimental evidence; yellow, text-mining

evidence; black, co-expression evidence. B) The KEGG pathway showing retinol metabolism was

significantly increased in the livers of patients with NAFLD. Proteins found in our comparative proteomic

study were labeled in red.

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Figure S4. Tissue distribution and adenovirus-mediated hepatic overexpression of 17β-HSD13 in

C57BL/6 mice. A) Tissue distribution of 17β-HSD13 in male and female C57BL/6 mice.17β-HSD13

mRNA levels were determined by real-time PCR in various mouse tissues. 17β-HSD13 was predominantly

expressed in the liver and appeared to be higher in female mice than male mice. *p<0.05, n=3. B)

Validation of the recombinant 17β-HSD13 adenovirus in HepG2 cells. Western blot analysis shows a dose-

dependent expression of 17β-HSD13 protein. C) Adenovirus-mediated overexpression of 17β-HSD13 for 4

days markedly increased 17β-HSD13 protein levels in liver. D) Hepatic overexpression of 17β-HSD13 had

little effect on serum TG and CHO levels.

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Figure S5. Overexpression of 17β-HSD13 increased the size of LD in both HepG2 and Huh7 cell

lines. A) HepG2 cells transfected with a GFP expression vector (GFP) or a 17β-HSD13-GFP construct

were visualized by confocal microscopy. Green: 17β-HSD13-GFP or GFP; Mauve: Nile red; Blue:

Nuclear. B) Huh7 cells were transfected with GFP or 17β-HSD13-GFP for 24 h. The size of the purified

LDs was measured by a Delsa Nano C particle analyzer (Beckman). The size of LDs was distributed in the

range of 50 nm to 3,000 nm. C) The diameters of LDs were larger in 17β-HSD13-GFP transfected cells

than that of GFP. *p<0.05, **p<0.01 vs. control (GFP), n=5.