2H + H+ OXALOACETATE PYRUVATE SUCCINYL-CoA GLUTARATE CITRATE MALATE O X O - 2 - ASPARTATE NO 2 - NO 3 - N 2 NH 4 CH3COSCoA ACETYL-CoA HOH CO2 NAD T R A N S A M I N A TI O N 2H + 2H + + CO2 5-Amino- levulinate Glycine H+ H+ H+ H+ H+ H+ c H+ H+ F0 F1 F1 β 2 1 α β α γ c -sub - u n it s A D P + P i 3. 6. 1.3 4 H + -tra n sp ortin g A T P s y n t h a s e α δ F6 oscp ATP + P D A i P FUMARATE UQH2 UQ UQH2 NADH+H + CH2COO - C(OH)COO - CH2COO - CH(OH)COO - CHCOO - 2COO - -OOCCOCH2CH2COO- -OOCCH2CH2CO.SCoA - OOCCOCH2COO- CH3CH(OH)CH2CO.SCoA - OOCCH=CHCOO - Glyoxylate Cycle -OOCCHO GTP GDP ATP + HEME Protoporphyrinogen Coproporphyrinogen Uroporphyrinogen Porphobilinogen 5-Amino- levulinate COO- CH2 CH2 H2NCH2C=O FADH 2 FAD Cyt.b Fe-S SUCCINATE II CH3COCOO- PYRUVATE Pi X Y Cyt.c1 Fe-S 2e- 2e- 1e- 1e- III 2UQ UQ UQH2 2UQH2 2UQ. _ UQ. _ Cyt.b L Cyt.b H Cyt.c IV Cu A Cu B Cu A 2e- Heme a Heme a 3 2H + 2H + 4H + 4H + 2H + NAD+ NADH+H+ NAD+ 1.1.1.39 1.2.4.1 2.3.1.12 3.1.3.43 4.1.1.32 4.1.3.7 4.2.1.3 1.1.1.41 1.2.4.2 2.3.1.61 6.2.1.4 -OOCCH2CH2COO- 1.3.5.1 4.2.1.2 1.1.1.37 6.4.1.1 4.1.3.8 4.1.3.1 4.1.3.2 ISOCITRATE 2.6.1.1 6 1 . 1 . 3 . 2 4.1.1.71 6 1 . 1 . 2 . 1 4.3.1.1 1.4.1.2 1.4.1.14 6.3.5.4 1.6.6.1 1.7.99.4 1 . 7 . 7 . 1 4 . 6 . 6 . 1 1 . 6 . 8 1 . 1 2.3.1.37 1.10.2.2 1.10.2.2 γ ε δ β 3 3 β A T P ADP E N D ER G ONIC RE ACTI O N 3 . 5 . 6 . 1 I FMNH2 FMN I 1.6. 5.3 2Fe -S (5 Clusters) H+ H+ H+ H+ ATP 4H + 2H + 2H + or H+ PHOTO- SYSTEM l O 2 3.6.1.34 NADP + Glyceraldehyde Ribulose-1,5-bis-P 2 β 8 8 H + c 3 2 ε a 3 2 α 1 α ε α β 2 1 3 α α β 3 α H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H + H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ H+ Translocated protons H+ H+ H+ H+ P i Ferredoxin 4H+ PC PC PC PQH2 2PQH2 PQ _ . 2PQ _ . Fe-S Cyt.f PQ 2PQ Cyt bf 2e- 1e- 1e- 2e- 2e- 2e- 2e- C yc lic Photo ph o sp ho ryla tion PQ PHOTO- SYSTEM II Cyt bc N o n - cy c l ic e le c tr o n flo w 2e- 2e- Mn 2e- * Protons from Water H 2 O H+ H+ 2H + 2H+ γ CO 2 Fixation H+ ADP Pi H+ NADPH+H + ATP THYLAKOID MEMBRANE CHLOROPLAST OUTER MEMBRANE THYLAKOID LUMEN STROMA ( ele c tric curr e nt) P680 Chl.a QA QB Pheophytin P700 Chl.A0 A1 Fe-S ATP ADP ATP 1 / 2O2 H 2 O PQH2 To Brain - A M I N O A C I D S P Y R I M I D I N E S P U R I N E S C A T E C H O L A M I N E S A R O M A T I C A M I N O A C I D S hv VISION L I P I D P H O T O S Y N T H E S I S P O R P H Y R I N S S T E R O I D S I S O P R E N O I D S P H O S P H O L I P I D S D E G R A D A T I O N L I P I D B I O S Y N T H E S I S P E N T O S E S H E X O S E S P O L Y S A C C H A R I D E S CO CH CH(NH )COO 2 3 + FOLIC ACID C1 POOL VALINE ATP ATP 4.1.1.4 1.1.1.30 CH COCH 3 3 CH COCH COO 3 2 CH CH(OH)CH COO 3 2 3-OH-Butyrate Acetone Acetoacetate KETONE BODIES HYALURONIC ACID DERMATAN BLOOD GROUP SUBSTANCES PEPTIDO- GLYCAN N-Ac-Neuraminate (Sialate) UDP- Iduronate UDP-N-Ac- Galactosamine UDP- Galacturonate GDP-Fucose TDP-Rhamnose ADP- Glucose UDP-Glucose TDP-4-Oxo- 6-deoxyglucose MANNOSE CHITIN CHONDROITIN PECTIN ALGINATES INULIN CELLULOSE O-ANTIGENS STARCH GLYCOGEN LACTOSE GALACTOSE O OH HO OH OH CH 3 O OPPT O OH OH O OH ACNH HO OH O OH HO OPPU NHAC OH OH OH OH HO OH NHCOCH OH HO O OPPG CH OH 2 HO P UDP-N-Ac- Glucosamine pyruvate N-Ac-Mannosamine-6-P UDP-Glucuronate GDP-Mannose N-Ac-Mannosamine UDP-N-Ac-Glucosamine N-Ac-Glucosamine-6-P Sorbitol Fructose-1-P Erythrose-4-P 3-Dehydrogulonate L-Xylose L-Lyxose D-Xylulose D-Ribose Ribitol L-Arabitol L-Xylulose L-Ribulose L-Ribulose-5-P L-Xylulose-5-P L-Arabinose Xylitol D-Xylose D-Arabinose D-Ribulose ASCORBATE 2, 3-Dioxogulonate N-Ac-Glucosamine-1-P Inositol-P Inositol Glucuronate Gulonate Gulonolactone 2-Oxogulonolactone Fructose 6-P-Gluconate D-Ribulose-5-P D-Xylulose-5-P Mannose-1-P GDP-Glucose TDP-Glucose UDP-Galactose Galactose-P Glucose-1-P Glucose-6-P Fructose-6-P Fructose- 1:6-bis-P OH H H H O HOCH 2 C C C C CO OH OH H OH H H O HOCH 2 C C C CO CO OH H H OH H C C OH CO P-Ribosyl amine Glycerate 2, 3-Diphospho- glycerate 3-P-Glyceraldehyde 1:3-bis-P-Glycerate 3-P-Glycerate 2-P-Glycerate P-enolpyruvate Inositol CH OH 2 CH OH 2 HOCH CH OH 2 CH O 2 HOCH P Glycerol UDP-Galactose UDP-Sugars CH CH OH 3 2 ETHANOL Dihydroxy- acetone-P (Glycerone-P) Glucosamine-6-P Phospho- serine Chain elongation Mitochondrial Serine Serine Indole-3-glycerol-P 3-Deoxy-D-arabino- heptulosonate-7-P Indoleacetate (Auxin) Indoxyl Formylkynurenine Kynurenine 3-Hydroxy kynurenine 3-Hydroxy anthranilate 2-Amino-3-carboxy muconate semialdehyde NH OH Tryptamine 1-(o-Carboxy phenylamino) 1-deoxyribulose-5-P N-(5-P-Ribosyl) anthranilate Anthranilate Catechol NH Indole- acetaldehyde NH CH CHO 2 Indolepyruvate N H C-CH(OH)CH(OH)CH O 2 P CH 2-Aminomuconate- 6-semialdehyde CH CH NH 2 2 2 NH 1.2.3.7 4.1.1.43 3.5.1.9 3.7.1.3 4.2.1.20 1.13.11.6 4.1.1.45 2.4.2.18 4.6.1.4 4.1.1.48 1.14.13.9 4.6.1.3 Dehydro- quinate 4.2.1.10 1.1.1.25 2.5.1.19 Shikimate-3-P Shikimate-5 enolpyruvate 3-P Chorismate PEP 2.7.1.71 9 1 . 1 . 4 . 1 Homogentisate Phenylpyruvate Cinnamate Coumarate Prephenate 2-Amino muconate Fumarate Aspartate GLYCINE Sarcosine Hydroxy- pyruvate P-Hydroxy- pyruvate SERINE Imidazole glycerol-P P-Ribosyl-ATP P-Ribosylformimino 5-aminoimidazole- carboxamide-R P Formimino glutamate Imidazolone propionate Urocanate Cystathionine Homocysteine Phosphoadenylyl- sulphate (PAPS) CH (NH )COOH 2 3 + Histidinol-P Histidinol Histidinal 3.1.3.15 2.6.1.9 1.1.1.23 3.6.1.31 5.3.1.16 2.4.2.17 Asparagine ALANINE 3-Sulphinyl pyruvate Oxobutyrate 2-Aceto-2- hydroxy- butyrate O-Phospho- homoserine HO SCH CH NH 2 2 2 2 Hypotaurine HO SCH CH NH 3 2 2 2 Taurine Glutamate γ-Glutamylcysteine Glutathione Glycine Cysteine ß-Alanine Cysteate S-Adenosyl homocysteine 4.2.99.2 1.1.1.86 4.2.1.9 1.1.1.86 4.2.1.9 2.6.1.32 8 1 . 3 . 1 . 4 S-Adenosyl methionine (SAM) 2-Methylaceto- acetyl-CoA 2-Acetolactate 2-3-Dihydroxy isovalerate 2-Oxo- isovalerate 3-Hydroxy- isobutyrate 3-Hydroxy- Isobutyryl-CoA Methyl acrylyl-CoA 2-Methyl-3- hydroxy- butyryl-CoA Tiglyl-CoA 2 Methylbutyryl- CoA 2:3-Di-OH- 3-methylvalerate 2-Oxo-3-methyl valerate Isobutyryl-CoA Cysteine sulphinate 4.2.99.9 2-Isopropyl- malate CH CH COCOO 3 2 + CH CH(NH )COO 3 3 CH CH(OH)COO 3 HO SCH COCOO 2 2 OCH COCOO 2 P OCH CH(NH )COO 2 3 P HOCH COCOO 2 HOCH CH(NH )COO 2 3 + + OH CH COCOO 2 COO OH OH O COO OH OH HO P COO OH OH O CH2 P COO O-C-COO OH O OH OOC CH COCOO 2 N H HOC-CH(OH)CH(OH)CH2OP CH COO NH 2 COO OH NH 2 COO COO OCH OOCCH NHCH 2 3 CH (CH ) COS-ACP 3 2 14 CH (CH ) COSCoA 3 2 14 CH (CH ) CH=CHCO-S-ACP 3 22 CH CH=CHCO-S-ACP 3 CH CH(OH)CH COS-ACP 3 2 COSCoA Linoleate Oleoyl-CoA Stearoyl-CoA Dehydrostearoyl-CoA OH-Stearoyl-CoA Oxostearoyl-CoA Palmitoyl-ACP Palmitoyl-CoA ACYL-ACP 2, 3-Enoyl-ACP 3, 4-Decenoyl-ACP 2, 3-Decenoyl-ACP 3-OH-Acyl-ACP 3-Oxoacyl-ACP 3-Oxo-Decanoyl-ACP 3-Oxo-Hexanoyl-ACP 3-OH-Hexanoyl-ACP 2, 3-Hexenoyl-ACP Butanoyl-ACP Crotonoyl-ACP 3-OH-Butanoyl-ACP Acetoacetyl-ACP Hexanoyl-ACP 3-OH-Decanoyl-ACP Decanoyl-ACP Palmitoleoyl-ACP γ-Linolenate Arachidonate Leukotriene B4 COSCoA CO-S-ACP Thromboxane B2 HOOCCH CO-S-ACP 2 Malonyl-ACP HOOCCH CO-SCoA 2 Malonyl-Co-A CH CO-S-ACP 3 Acetyl-ACP CH O-CO-R 2 CH OH 2 R’-CO-OCH FATTY ACID ACYL-CoA (Cytosol) CH O-CO-R 2 CH O 2 R’-CO-OCH P Cardiolipin Phosphatidylglycerol Acetylcholine Glycerophosphocholine Lysolecithin Choline plasmalogen CDP-choline Choline-P Mevaldate CH (CH ) CH=CHCH(OH)CHCH OH 3 2 12 2 Dehydrosphingosine Sphingosine 4-Sphingenin Psychosine Acyl-CoA Acyl-CoA Cerebroside Galactose CH (CH ) CH=CHCH(OH)CHCH O- 3 2 12 2 NHCOR Isopentenyl-PP (C5) Dimethylallyl-PP (C5) Geranyl-geranyl-PP (C20) Farnesyl-PP (C15) Squalene (C30) Geranyl-PP (C10) Desmosterol Zymosterol Lanosterol CH C = CHCH O 3 2 PP CH 3 Ceramide Gangliosides NHCOR CH O 2 PP OPP CH 2 CHOLESTEROL CH C(OH)CH CH OH 3 2 2 CH COO 2 CH C(OH)CH CHO 3 2 CH COO 2 NADP + Dehydroascorbate OH H O HOCH 2 C C C C CO OH H OH OH H O HOCH 2 C C CO CO CO H NAD+ Pi ADP ADP Phytoene (C40) Lycopene (C40) ß-CAROTENE (C40) Rhodopsin Metarhodopsin Retinoate trans-Retinal 11-cis-Retinol trans-Retinol (Vitamin A) Retinol esters Dark Light Ubiquinone (Coenzyme Q) Menaquinone Plastoquinone Phylloquinone (Vitamin K) Opsin CHO CH OH 2 CHO CH OH 2 CH 3 CH O 3 CH O 3 n O O O O Phytol (C20) α-Tocopherol (Vitamin E) Quinolinate Quinolinate- nucleotide Nicotinate- nucleotide Desamino-NAD 5-Hydroxy- tryptophan 5-Hydroxytryptamine (SEROTONIN) N-Acetyl-5-O-methyl-serotonin (MELATONIN) NH CH CH NHCOCH 2 2 3 HO NH CH CH NHCOCH 2 2 3 CH O 3 + NAD( P) NICOTINATE N-Acetyl-serotonin 2.4.2.19 Dopamine Dopa Dopaquinone THYROXINE MELANIN OH OH CHOHCH NHCH 2 3 OH OH CHOHCH NH 2 2 4-OH-3-Methoxy- phenylglycol CHOHCH NH 2 2 OH OCH 3 OCH 3 CHOHCH OH 2 OH OCH 3 4-OH-3-Methoxy- D-mandelate Cyclic AMP 2H-folate 4H-folate ATP Dephospho-Coenzyme A 4-P-Pantetheine 4-P-Pantothenylcysteine 4-P-Pantothenate Pantoate 6.3.2.1 1.1.1.169 3.5.1.22 2.7.1.33 NH N H O O O O CH OH 2 OH OCH 3 CH(OH)COO H2 NH 3 + CH-COO C O O COO N Ribose- P N + COO NH CH COO 2 O OH CH O 2 P NHCOCH NH 2 2 OH Formyl glycinamide-R P Urea Formyl glycinamidine-R P Allantoate Allantoin URATE ADP Xanthine Hypoxanthine Inosine Adenosine Adenylo- succinate 5-Amino imidazole-R P 5-Amino-4-imidazole carboxylate-R P 5-Amino-4-imidazole (N-succinylcarboxamide)-R P 5-Aminoimidazole carboxamide-R P Formylamido- imidazole- carboxamide-R P H NCONH 2 2 HN HC 2 CHO NH RP NH C C C O C CO OC NH NH H N HN H C C O C CH OC NH N N HN Carbamoyl ß-alanine Dihydro- uracil Uracil d-ADP d-ATP GTP GDP XANTHOSINE-P (XMP) TTP TDP ß-Ureido isobutyrate Dihydro thymine Thymine d-UMP d-CMP d-CDP CDP Carbamoyl aspartate Dihydro orotate Orotate Orotidine-P Uridine-P (UMP) UDP 3-Amino- isobutyrate CH C NH 2 CH OC NH N CH C O CH OC N H HN CH-CH 3 C O OC NH HN CH 2 CH 2 CH 2 C O OC NH HN C O CH OC NH HN C CH 3 Methylmalonyl semialdehyde OHCCHCOO CH 3 CH 2 C O CH-COO OC NH HN CH C O C-COO OC NH HN H NCONHCH CH COO 2 2 2 C C C CH RP HC NH NH OOC-CH-CH COO 2 N N N CHO NH RP NH OC HC 2 2.7.7.43 3.1.3.29 1.1.1.158 5.1.3.13 3.1.3.29 4.1.3.20 2.7.1.60 5.1.3.14 5.1.3.7 5.1.3.1 4 . 1 . 7 . 2 5.4.2.3 7 1 . 1 . 1 . 3 1.1.1.49 1 2 . 1 . 1 . 1 2.7.7.23 3.1.3.25 1.13.99.1 1.1.1.19 1.1.3.8 5.3.1.3 2.7.1.53 9 . 1 . 1 . 1 5 1 . 1 . 7 . 2 7 1 . 1 . 7 . 2 4 . 3 . 1 . 5 5.1.3.1 2.2.1.2 4.1.2.13 2.7.1.11 2.7.1.47 2.2.1.1 1.2.1.12 1.1.1.29 1.1.1.95 1.3.1.35 1.14.99.25 2.3.1.41 2.3.1.41 2.3.1.41 1.1.1.100 5.3.99.5 4.2.1.60 3 . 9 9 . 3 . 5 1 . 9 9 . 4 1 . 1 1.13.11.34 1.3.1.10 1.3.1.9 1.14.99.5 9 . 1 . 3 . 1 4.2.1.61 0 6 . 1 . 2 . 4 0 6 . 1 . 2 . 4 4.2.1.59 4.2.1.58 1.3.1.9 1.3.1.9 6.2.1.3 3.1.2.20 1.1.1.100 1 . 1 . 3 . 5 HOOC-COOH Oxalate Glycolate HOCH CHO 2 Glycol aldehyde Ethanolamine-P 2.7.1.30 2.3.1.7 2 . 1 . 7 . 3 4.1.3.5 2.7.8.8 2.1.1.17 2.1.1.71 1.3.1.35 2.7.8.2 3.1.4.3 3.1.4.4 2.7.7.15 5 . 1 . 1 . 3 2 . 4 . 1 . 3 2.7.1.32 . 1 . 7 . 2 2 8 1.2.4.1 2.3.1.12 1.8.1.4 2 . 1 . 6 . 2 . 1 . 4 . 1 1 1 . 1 . 1 . 4 2.3.1.50 1.1.1.102 3 . 8 . 7 . 2 2 6 . 1 . 4 . 2 3.2.1.46 5.2.1.3 1.2.1.36 2.3.1.76 3.1.1.21 5.2.1.7 5.4.99.7 1.14.99.7 4.2.1.75 1.3.3.3 4.99.1.1 2.5.1.29 4.1.1.28 2.3.1.5 2.1.1.4 6.3.5.1 6.3.1.5 1 1 . 2 . 4 . 2 2.7.7.18 2.6.1.5 1.2.1.32 1.13.11.5 2.1.1.28 2.1.2.2 6.3.3.1 3.5.2.5 0 1 . 1 . 4 . 1 1.7.3.3 1.1.3.22 4.1.1.28 4.1.1.21 2.1.2.3 2.4.2.1 1.5.99.2 2.1.1.5 2.6.1.22 3.5.2.3 2.6.1.51 1.4.1.7 3.1.3.3 2.6.1.52 2.1.3.2 ACETATE Glycine 4 . 2 . 4 . 2 2.1.1.45 RP CH C O CH OC N HN 1.3.1.14 2.4.2.10 4.1.1.23 7 . 7 . 7 . 2 7 . 7 . 7 . 2 2.7.7.7 . 7 1 . 1 1 . 4 7 . 7 . 7 . 2 2.7.4.14 4.4.1.1 1.6.4.1 1.13.11.20 4.4.1.8 4.2.1.22 7 2 . 1 . 1 . 1 4.1.1.29 3 . 2 . 3 . 6 2 . 2 . 3 . 6 1.8.1.3 LACTATE Acetaldehyde 2.7.1.24 2.7.7.3 4.1.1.36 Bile Acids CH CH COSCoA 3 2 Propanoyl-CoA 1 3 . 1 . 1 . 1 2.1.3.1 4.1.1.41 5.1.99.1 6.3.2.5 2.1.1.14 2.1.1.13 1 . 1 . 6 . 4 CDP-Ethanolamine 1.1.1.35 4.2.1.17 + HO SCH CH(NH )COO 2 2 3 3.5.2.7 NH HN CH OOC CHCH CH COO 2 2 CMP-N-Acetyl neuraminate CDP-diacyl glycerol CHOLINE Ethanolamine Glyoxylate HISTAMINE 2.3.1.46 Homoserine Tyramine Plant Pigments Tannins Maleyl acetoacetate Fumaryl acetoacetate 5.2.1.2 CH COO 2 OH OH OH CH CH NH 2 2 2 OH OH CH CH NH 2 2 2 Hydroxyphenyl pyruvate α-Tocopherol (Vitamin E) LIGNIN d-GTP OH OH OH O HO OH P OPPT CH 3 HO OH OH O NADPH 2.2.1.1 2.7.1.3 1.1.1.45 1.1.1.130 1.10.3.3 1.10.2.1 1.1.1.10 ATP CH CH NH 2 2 2 NH HO 5.4.99.5 2.6.1.5 1.13.11.27 Indole 4.1.99.1 1.13.11.11 OH O OH NH C C CH CH O 2 P H H COO NH 2 COO 6.3.5.3 6.3.2.6 4.3.2.2 Guanine DNA 6.3.4.4 4.3.2.2 C C O C CH HC NH N N HN C C O C CH HC NH N N HN 3.5.3.4 2 . 4 . 2 1 . 2.7.7.6 2.7.7.6 6 . 7 . 7 . 2 6 . 7 . 7 . 2 2.7.4.6 2.7.4.3 2.7.4.4 2.7.4.6 2.4.2.15 1.1.1.205 6.3.4.1 6.3.5.2 2.7.4.4 3 . 4 . 5 . 3 2 . 2 . 2 . 3 2.4.2.1 . 3 . 1 . 3 5 3.1.4.6 6.3.4.2 1.17.4.1 1 . 4 . 7 1 . 1 3.5.4.12 2.7.4.8 9 . 4 . 7 . 2 6 . 4 . 7 . 2 2.4.2.4 1.3.1.2 3.5.2.2 3.5.1.6 3.5.1.6 3.5.2.2 3.5.4.1 Diphospho- mevalonate Mevalonate 3-Oxopentanoyl-CoA 3-Oxoacyl-CoA 3-OH-Acyl-CoA 2, 3-Enoyl-CoA 2, 3-Hexenoyl-CoA 3-OH-Hexanoyl-CoA Acetoacetyl-CoA 3-OH-Butanoyl-CoA Crotonoyl-CoA Pentanoyl-CoA 3-OH-Pentanoyl-CoA 1.1.1.35 1.1.1.35 1.1.1.157 1.3.99.3 1.3.99.2 1.1.1.35 Odd C Fatty acids Succinylhomoserine CO 2 2-OXO ACID Glutamyl-P Glutamine UREA P-Creatine Creatine Creatinine Glycine CITRULLINE Glutamic semialdehyde 2-AMINO ACID Pyrroline-5- carboxylate Glyoxylate Pyruvate 4-Hydroxy- 2-oxoglutarate 4-Hydroxy- glutamate 3-Hydroxy- pyrroline- 5-carboxylate Putrescine H NCH CH CH CH NH 2 2 2 2 2 2 Spermidine Spermine N -Trimethyllysine 6 6 N -Trimethyl- 3-OH-lysine Carnitine Glutaryl-CoA Saccharopine 2-Aminoadipate semialdehyde 2-Aminoadipate 2-Oxoadipate Arginino- succinate Guanidoacetate Methylmalonyl-CoA 4.1.2.12 Aspartyl Semialdehyde 2, 3-Dihydro- dipicolinate 1 1 . 1 . 2 . 1 4.2.1.52 1.3.1.26 Piperideine- 2, 6-dicarboxylate N-Succinyl- 2-amino-6-oxo- pimelate 2.6.1.17 N-Succinyl-2, 6 diaminopimelate Diamino- pimelate 3.5.1.18 3-Methyl- glutaconyl-CoA (CH ) CHCH COSCoA 32 2 (CH ) CHCH COSCoA 32 2 3-Methyl- crotonyl-CoA Isovaleryl-CoA Oxopantoate HCHO OOCCH C = CHCOSCoA 2 CH 3 CH COCHCOSCoA 3 CH 3 CH CH=CHCOSCoA 3 CH 3 CH CH(OH)CHCOSCoA 3 CH 3 C(OH)CH(OH)COO CH CH 3 2 CH 3 CH = CCOSCoA 2 CH3 HOCH CHCOS-CoA- 2 CH 3 CH CHCO-SCoA 3 CH 3 C (OH)CH(OH)COO CH 3 CH3 CH CH CHCOSCoA 3 2 CH 3 CH C = CHCOSCoA 3 CH 3 ATP 4-Aminobutyrate (GABA) 1.3.99.7 1.5.1.2 1.14.11.2 2.6.1.39 1.2.1.31 1.14.11.8 2.5.1.16 2.5.1.22 4.1.3.16 2.6.1.23 1.5.99.8 1.5.1.2 6.4.1.4 4.2.1.33 2.6.1.6 1.1.1.85 Oxoleucine COOH (CH ) CHCHCH(OH)COO 32 S-Adenosylmethyl thiopropylamine (Decarboxylated SAM) PROLINE Coenzyme A CH C= CHCH 3 2 CH3 O CH CH 3 3 HO CH 3 CH 3 CH 3 CH C-CH HO 3 2 2 C PP CH 2 CH C= CHCH O 2 2 PP CH 3 3-P-Glycerol Phosphatidyl ethanolamine CEPHALIN 2 1 . 4 . 1 . 3 Progesterone 1.5.1.12 2.7.3.2 4.2.1.17 3.1.2.4 2.6.1.32 5 1 . 1 . 4 . 4 Acetylserine 2.7.7.4 2.7.1.25 1.8.99.1 Adenylylsulphate (APS) 2.7.1.39 5 1 . 1 . 4 . 4 9 2 . 1 . 1 . 4 8 . 9 9 . 2 . 4 0 3 . 1 . 3 . 2 P-Ribosyl-PP 1.1.1.1 1.2.1.4 Aspartyl-P 7 . 1 . 1 . 5 2.7.1.40 8 1 . 3 . 1 . 4 . 3 . 1 . 4 8 1 1.5.1.9 1.14.11.1 CHOLINE 1 . 1 . 1 . 2 3 1.3.1.2 NHCH CH SH 2 2 NHCH CH CO 2 2 OCH C(CH ) CH(OH)CO 2 32 P ADP- NHCH CH SH 2 2 NHCH CH CO 2 2 OCH C(CH ) CH(OH)CO 2 32 P-ADP- NHCH CH SH 2 2 NHCH CH CO 2 2 OCH C(CH ) CH(OH)CO 2 32 1.4.1.8 1.3.99.3 1.3.99.3 1.4.1.9 1 . 1 . 2 . 4 8 . 1 . 1 . 4 0 2 1.3.99.7 1.8.99.2 + CH CO-OCH CH(NH )COO 2 2 3 2.5.1.6 2.1.1.10 2.1.1.20 1.1.1.3 1.1.1.3 4.2.1.18 4.1.2.5 5 . 1 . 3 . 4 0 2 . 1 . 2 . 4 0 2 . 1 . 1 . 2 Glyoxylate 6 . 1 . 1 . 2 4 . 3 . 4 . 1 Hexanoyl-CoA Butanoyl-CoA 6.3.5.5 1 1 . 2 . 7 . 2 NO 1.14.13.39 3.5.3.6 3.5.3.1 4.1.1.17 2.7.7.41 4 . 1 . 6 . 2 1.1.1.100 1.1.1.8 1 5 . 1 . 3 . 2 . 1 . 3 . 2 5 1 2.7.8.5 SO - 4 2 1.14.16.4 NH 2 OOC OOC 5 2 . 1 . 1 . 4 1.3.1.13 1.3.1.13 2.7.4.6 CYTIDINE- triphosphate (CTP) Cytosine 2.4.2.9 4.1.1.11 2.6.1.18 4.3.1.3 4.2.1.49 3 HSO - 1.1.1.105 2.3.1.41 NADPH 2 2 . 1 . 1 . 1 . 1 . 3 . 2 4 5.5.1.4 3.2.1.26 3.2.1.48 2.6.1.16 1.1.1.14 2.7.7.34 4.2.1.47 2.4.1.68 2.4.1.69 2.4.1.9 5.3.1.8 2.4.1.11 O OPPU CH OH 2 CH OH 2 HO OH OH HO 2.4.1.21 2 5 . 1 . 2 . 4 1.2.1.18 8 1 . 1 . 2 . 4 O CH C CH OC N HN DP CH C O CH OC N HN RPPP OCH 2 H C C H OH OH CH N HN C H C P H O OCH 2 C C H OH H OH C H C C C NH 2 C CH HC N RP(PP) N N N + P NH 2 H O OCH 2 C C H OH H OH C H C C OC C CH HC N RP N N NH P H OCH 2 C C H OH OH CO CH 2 C CONH 2 C CH HC N RP N N NH P + OOCCH N(CH ) 2 33 Betaine Betaine aldehyde 2.4.1.16 5.1.3.6 2.4.1.33 2.7.7.13 5.4.2.8 5.3.1.8 O OH CH OH 2 HO OP HO 2.7.1.28 2.7.1.31 6.3.4.13 1 . 1 . 3 . 5 2.2.1.1 4.1.1.9 3.1.2.11 ß-OH-ß-Methyl- glutaryl-CoA 6.3.4.5 5.3.1.6 2.5.1.21 2.1.1.2 3.5.2.10 6.3.2.13 6.3.2.7-10 2.7.7.27 2.7.7.9 3.5.4.10 C C O C CH OC NRP N N H HN C C O C CH N N N HN RP HN 2 C 2.7.4.6 1.17.4.1 2.7.4.6 2.6.1.13 Carbamoyl-P 9 . 1 . 3 . 2 Carnitine 4 . 1 . 6 . 2 4 4 . 1 . 6 . 2 CH C(OH)CH COSCoA 3 2 CH COO 2 1.2.3.5 1.1.1.34 6 1 . 1 . 3 . 2 4.1.3.5 1 . 1 . 3 . 2 6 Menaquinone 1 . 1 . 1 . 4 5 4.2.1.16 Carnosine CH CH NH 2 2 2 C NH N C HC H 4.1.1.22 4.3.1.3 Ubiquinone Plastoquinone D-Ribose-5-P NH CH CH(NH )COO 2 3 HO + O OH OH HO OPPU COO - O OH OH HO OPPU COO - OH H OH HOCH 2 C C CO H CO COO - O CHOH CHOH AcNH HO OPC O CO - CH OH 2 O CHOH CHOH AcNH HO OH COO CH OH 2 O OH OH HO HO CH O 2 P O OH ACNH HO OH CH O 2 P O O OH HO HO P CH OH 2 O OH OH HO HO CH OH 2 O OH HO OH 3 CH O 2 P O OH HO O NHCOCH 3 P CH OH 2 CH OH 2 CH OH 2 O OH HO OH NH2 CH O 2 P O OH HO OH OPPU CH OH 2 O O HO OH OH HO P CH OH 2 CH OH 2 O O HO OH OH O OH OH OH CH OH 2 O OH HO OH OH CH 2 CH OP 2 OH H H H OH C C C C OH OH H COO - HOCH 2 H OH OH OH C C C CHO H H HOCH 2 H OH OH H C C C CHO H OH HOCH 2 OH H OH H C C C CHO H OH HOCH 2 H OH H OH C C C CHO OH H HOCH 2 OH H H OH C C C CHO OH H HOCH 2 OH H OH OH C C C CHO H H HOCH 2 H OH H OH C C C C OH H H OH COO - POCH 2 OH H OH C C CO H POCH 2 CH OH 2 H OH H C C CO OH HOCH2 CH OH 2 CH OH 2 POCH 2 H OH OH C C CO H CH OH 2 POCH 2 OH H H C C CO OH CH OH 2 POCH 2 H OH OH C C CO H HOCH 2 CH OH 2 OH H OH C C CO H HOCH 2 CH OH 2 OH H H C C CO OH HOCH 2 CH OH 2 H OH OH H C C C H OH HOCH 2 CH OH 2 OH H OH OH C C C H H HOCH 2 CH OH 2 H OH H OH C C C C OH H H OH CH OH 2 HOCH 2 H OH OH C C CHO H POCH 2 H OH H OH C C C CO OH H HOCH 2 CH O 2 P H OH OH OH C C C H H HOCH 2 CH OH 2 H OH OH C C C H CO H HO POCH 2 CH OH 2 P H OH OH C C C H CO H HO CH O 2 P OCH 2 H OH OH C C H C CHO OH H POCH 2 POCH 2 O OH NH 2 OH CH O 2 P CHOHCHO POCH 2 HOCH 2 COCH 2 OP O OH O O P P OH CH O 2 P CHOHCOOP POCH 2 CHOH POCH 2 COO COO OH O HO OH COO CH (CH ) CH(OH)CH COS-CoA 3 2 14 2 CH (CH ) CH(OH)CH COS 3 2 14 2 CH (CH ) COCH COS-CoA 3 2 14 2 CH (CH ) COCH COS-CoA 3 2 14 2 CH (CH ) COS-CoA 3 2 n+2 CH (CH ) CH=CHCOS-CoA 3 2 14 CH (CH ) 3 2n CH=CHCOS-CoA CH (CH ) 3 25 2 CH=CHCH COSACP CH (CH ) 3 26 CH=CHCOSACP CH (CH ) 3 26 COCH COSACP 2 CH (CH ) 3 26 2 2 CH CH COSACP CH (CH ) 3 22 2 2 CH CH COSACP CH CH CH COSACP 3 2 2 CH (CH ) 3 26 CH(OH)CH COSACP 2 CH CH=CHCO.S-ACP 3 CH (CH ) 3 22 2 COCH COSACP CH 3 COCH COSACP 2 CH (CH ) 3 2n 2 COCH COSACP CH (CH ) 3 2n 2 CH(OH)CH COSACP CH (CH ) 3 22 2 CH(OH)CH COSACP ACYL-CoA (Mitochondria) CH (CH ) CH CH COSCoA 3 2 2 2 n CH (CH ) CH=CHCOSCoA 3 2 n CH (CH CH(OH)CH COSCoA 3 2 n CH (CH ) CH CH COSCoA 3 22 2 2 CH (CH ) CH=CHCOSCoA 3 22 CH (CH ) COCH COSCoA 3 2 2 n CH COCH COSCoA 3 2 CH (CH ) CH(OH)CH COSCoA 3 22 2 CH CH(OH)CH COSCoA 3 2 CH CH=CHCOSCoA 3 CH CH CH COSCoA 3 2 2 CH CH CH CH COSCoA 3 2 2 2 Pentenoyl-CoA CH CH CH=CHCOSCoA 3 2 CH CH CH(OH)CH COSCoA 3 2 2 CH (CH ) COCH COSCoA 3 22 2 CH CH COCH COSCoA 3 2 2 OH OH HO OH O O- CH O-PO 2 CH CHOHCH OH 2 2 O CH 2 CH 2 R'-CO-OCH O-CO-R O-PO O- HOCH CH 2 2 NH 3 + POCH CH 2 2NH 3 + CPP- OCH CH 2 2NH 3 + O - CH O-CO-R 2 CH O P 2 OCH CH 2 2NH 3 + CH COCH CH N(CH ) 3 2 2 33 HOCH CH N(CH ) 2 2 33 + CH CH 2 COO (NH ) 3 + OH OH CH CH 2 COO- (NH ) 3 + CO NH 2 CH CH(NH )COO 2 3 + NH OC CO NH OC N H C H HN 2 HC CH N N C RP HN 2 CH C CH OC N N DP NH 2 RPPP CH C CH OC N N NH 2 C C C CH RP(P) HC N N N N NH 2 H NCOO 2 P (CH ) NH 23 2 H N(CH ) NH 2 23 (CH ) NH 24 (CH ) NH 23 2 H N(CH ) NH 2 24 CH -SCH CH CHNH 3 2 2 2 Adenosyl + + CH 3 CH 3 CHCH(NH )COO 3 + C NH N C HC H CH CH(NH )CH OH 2 3 2 + C NH N C HC H CH CH(NH )CH OP 2 3 2 C NH N C H HC CH COCH OP 2 2 + HSCH CH CH(NH )COO 2 2 3 + + SCH CH CH(NH )COO 2 2 3 CH CH(NH )COO 2 3 RP C O C-COO OC N HN CH 2.4.1.23 CH (CH ) CH=CHCH(OH)CHCH OH 3 2 12 2 NH 3 + CH (CH ) CH(OH)CHCH OH 3 2 14 2 NH 3 + CH (CH ) COCHCH OH 3 2 14 2 NH 3 + Galactose CH (CH ) CH=CHCH(OH)CHCH O- 3 2 12 2 NH 3 + 4.1.1.70 H NCONH 2 2 OHCCH N(CH ) 2 33 + C-CH3 C O CH OC N DP HN DP CPP-OCH CH N(CH ) 2 2 33 + OCH CH N(CH ) 2 2 33 + 1.2.1.41 UDP-N-Ac-Muramate O O CH CH 3 COO- NHAC HO OPPU CH OH 2 4.1.3.20 O OH CH 3 HO HO OPPG 2.7.7.24 2.4.1.22 2.7.7.12 5.1.3.2 4 . 1 . 3 . 5 Sedoheptulose-PP 3.1.1.28 4.2.1.24 N CH 2 CH 2 N N N CH 3 CH 3 CH CH COO CH CH CH 2 HC 3 HC 3 HC 3 HC C C H H Fe CH 2 CH 2 COO - - CH 3 CH 3 CH 2 CH 2 CH 2 HC 3 HC 3 N H N H N H N H CH CH HC 2 C C H 2 H 2 - CH 2 CH 2 COO CH 2 CH 2 COO - HC 2 HC 2 CH2 CH 2 OOC COO HN 2 N H - - CH 2 HC 2 HC 2 HC 2 H 2 H 2 CH 2 CH2 CH 2 CH2 CH 2 CH 2 N H N H N H N H COO COO COO COO C C - - CH 2 CH 2 COO CH 2 CH 2 COO - - - - OOC - OOC - CH 3 CH 3 HC 3 HC 3 HC 2 H 2 H 2 N H N H N H N H CH2 CH 2 CH 2 CH 2 CH 2 C C COO - CH 2 CH 2 COO CH 2 CH 2 COO - - COO - COO - CH C(OH)CH CH O 3 2 2 PP CH COO 2 OHCCOO HOCH COO 2 NH 2 H NCNHCH COO 2 2 + NH 2 H NCN(CH )CH COO 2 3 2 + NH 2 HNCN(CH )CH COO 3 2 P- + HN C NH CO N CH2 CH 3 OOCCHCH COO 2 H NCNHCH CH CH CH 2 2 2 2 N (NH ) 3 + COO CHCOO HC CH 2 HOCH N OOCCH(OH)CH CH(NH )COO 2 3 + CHCOO CH 2 HOCH HC 2 N H NH CHCOO CH 2 CH 2 CH 2 OOCCH(OH)CH COCOO 2 N CHCOO CH 2 CH 2 CH CH COCOO 3 OHCCOO + H NCONHCH CH CH CH 2 2 2 2 (NH ) 3 COO H NOCCH CH CH 2 2 2 COO (NH ) 3 + R-CO-COO + COO R-CH(NH ) 3 + COO H NOCCH CH 2 2 (NH 3 + OOC-CH-COSCoA CH3 P OOCCH CH 2 COO (NH ) 3 + OOCCH CH CHO 2 2 OHCCH CH 2 COO (NH ) 3 + (CH ) NCH CH(OH)CH COO 33 2 2 + OHCCH CH CH 2 2 + COO (NH ) 3 POOCCH CH CH 2 2 + COO (NH ) 3 OHCCH CH CH CH 2 2 2 COO (NH ) 3 + NH CHCH CH COO 2 2 COO CH CH CH CH CH 2 2 2 2 COO (NH ) 3 + H N(CH ) 2 4 2 CH(NH )COO 3 CH(NH 3 + (CH ) N(CH ) 33 23CH CH(NH )COO 2 3 + + CH2 C H OOCC N HC CH-COO CH2 C H 2 CH-COO OOCC N HC 2 OOCCOCH CH CH CH-COO 2 2 2 CH CH 2 2 2 OOCCH CH CONH 2 2 OOCCHCH CH CH CH-COO 2 2 2 OOCCH CH CONH 2 2 CH 2 2 NH 3 + OOCCH-CH CH CH CHCOO 2 2 2 NH 3 + NH3 + (CH ) CHC(OH)CH 32 2 COOH COO (CH ) CHCH COCOO 32 2 + (CH ) CHCH 32 2 CH(NH )COO 3 HOCH CHCOO 2 CH3 CHCOCOO CH 3 CH 3 CH COC(OH)CH 3 3 COO + POCH CH CH(NH )COO 2 2 3 + HOCH CH CH(NH )COO 2 2 3 + CH CH CH(NH )COO 2 2 3 OOCCH CH 2 COO 2 + CH SH 2 OOCCH(NH )CH CH CONHCHCONHCH COO 3 2 2 2 HOCH C(CH ) COCOO 2 32 HOCH C(CH ) CH(OH)COO 2 32 NHCH CH COO 2 2 HOCH C(CH ) CH(OH)CO 32 ) 2 32 NHCH CH COO 2 2 OCH C(CH ) CH(OH)CO 2 32 P CH CH 3 2 CH 3 CH + CH(NH )COO 3 CH COC(OH)CH CH 3 2 3 COO CHCOCOO CH CH 3 2 CH 3 NHCHCH SH 2 COO NHCH CH CO 2 2 OCH C(CH ) CH(OH)CO 2 32 P + CH SH 2 OOCCH(NH )CH CH CONHCHCOO 3 2 2 + Adenosyl SCH CH CH(NH )COO 2 2 3 + SCH CH CH(NH )COO 2 2 3 + HO SCH CH(NH )COO 3 2 3 + + S-CH CH(NH )COO 2 3 S-CH CH(NH )COO 2 3 + HSCH CH(NH )COO 2 3 1.1.1.23 H C NH N C HC CH CHCOO 2 NHCOCH CH NH 2 2 2 + H C NH N C HC CH CH(NH )COO 2 3 CHCH CH COO 2 2 NH N CH OC C CH CHCOO NH N CH CH CH COO 3 N H OOC CH-COO OC NH 2 CH2 H NCH CH COO 2 2 2 H NCH 2 2 CH 3 CHCOO H NCONHCH CHCOO 2 2 CH 3 C C O CH RP HC N N N HN C C C O CH HCO N N N RP C CH N N HN 2 H C C C O CH N N C C CH N N RP HN 2 HN 2 C O O OP~ ~ P O O O O PO O CH2 N N N N O OH OH NH2 HC CH N N N N O O P O O O CH2 OH NH 2 HC CH COO CO NH OC N H C H HN 2 NH 2 OOC-CH-CH COO 2 HNCO C CH N N C RP RP HN 2 OOC C CH N RP N C HN 2 CH=CHCOO OH CH=CHCOO CH COCOO 2 CH2 P COO OH O O O COO O CH COO 2 O CH COO 2 -OOC OCH 2 CH 2 OH H C P HOCH HCOH OC COO COO HO OH OH O NH2 COO OCH NH CH COCOO 2 NH CHO CO NH 2 OH CH CH(NH )COO 2 3 + N COO COO N + COO COO RP O OH OH HO OPPU COO O OH NHCOCH 3 HO OPPU COO CH OH 2 O O C CH 2 NHAC HO OPPU COO CH OH 2 O OH HO OH OH COO OOCCH N(CH ) 2 32 Dimethylglycine 1.1.99.1 1.2.1.8 COO HO HO COO O HO OH COO CH(O ) P POCH2 COO CH(OH) HOCH 2 COO COO CH(O ) P HOCH 2 COO CH =C(O ) 2 P COO CONH 2 Ribose -O - P - O - P - O- Adenosine(P) + N O O O O + COO N Ribose - O - P - O - P - O -Adenosine O O O O I I I I OH O CH CH 2 COO (NH ) 3 + Epinephrine (Adrenaline) Norepinephrine (Noradrenaline) Normetepinephrine (Normetadrenaline) HOCH CH N(CH ) 2 2 33 + Adenosyl + + SCH CH CH(NH )COO 2 2 3 CH 3 (CH ) N(CH ) 33 23CH CH(NH )COO 2 3 + + OH OOCCH CH CH COSCoA 2 2 2 OOCCH CH CH NH 2 2 2 2 OOCCH CH CH COCOO 2 2 2 OOCCH CH CH CH 2 2 2 COO COO (NH ) 3 + HO H HO H H HO H HO H Prostaglandin PGE 2 OH HOCH 2 C C CO C COO OH H OH H H - COO O R'-CO-OCH CH O-CO-R 2 CH O POCMP 2 O CH CH N(CH ) 2 2 3 + O HOCH CH OPO 2 CH OH 2 O- CH OPO 2 O CH OCH=CHR 2 CH CH N(CH ) 2 2 33 + O- CH OPO 2 O R'-CO-OCH CH O-CO-R 2 3.1.1.32 CH CH N(CH ) 2 2 33 + O- O HOCH CH OPO 2 CH O-CO-R 2 CH CH N(CH ) 2 2 33 + O- CH CH(NH )COO 2 3 + + C NH N C HC H CH CH(NH )CHO 2 3 HS 11-cis-Retinal Pi ADP NADP + GDP- Mannuronate Mannose-6-P Dehydro- shikimate Shikimate RNA d-GDP d-CTP GUANOSINE-P (GMP) ß-Alanine P-Ribosyl-AMP P-Ribulosylformimino 5-aminoimidazole- carboxamide-R P Imidazole acetol-P HS HSO - PANTOTHENATE 3-Isopropyl- malate Succinic semialdehyde 2.4.1.17 Diacyl glycerol 2.3.1.6 1.14.12.1 1.4.4.2 CH CHO 3 3.5.4.19 4.2.1.19 3.3.1.1 4.1.1.12 4.1.2.5 COO- 2 OHCCH 2.1.4.1 2.4.99.7 5.1.3.12 1.1.1.132 4.2.1.46 2.7.1.7 2.4.1.29 2.4.1.21 2.4.1.13 5.4.2.2 2.7.1.6 2.7.7.10 3.1.1.18 4.1.1.34 2.7.1.47 2.7.1.16 ADP P-Glucono lactone 1.1.1.44 O O OH HO OH CH O 2 P PHENYLALANINE TYROSINE TRYPTOPHAN INOSINE-P (IMP) THYMIDINE-P HISTIDINE URIDINE- triphosphate (UTP) CYSTINE CYSTEINE ISOLEUCINE LEUCINE LYSINE ORNITHINE ARGININE HYDROXY PROLINE PHOSPHATIDYL SERINE Phosphatidyl inositol LECITHIN SPHINGOMYELIN STEROIDS Pregnenolone 2.7.6.1 2.4.2.14 6.3.4.7 5.3.1.9 3.1.3.11 1.2.1.13 2.7.2.3 H OH OH C C C H CO OH C H H OH POCH 2 CH O 2 P 5.4.2.1 ADP 4.2.1.11 Endoplasmic Reticulum 1.1.1.100 3.1.1.3 Phosphatidate 2.3.1.39 4.2.1.17 4.2.1.17 4.2.1.55 1.1.1.79 1.2.1.21 1.4.3.8 1.1.1.32 2.7.1.36 2.7.4.2 4.1.1.33 2.5.1.1 2.5.1.10 2.7.8.5 2.7.8.1 4.1.1.65 2.7.7.14 1.3.99.7 PO O CH (CH ) CH=CHCH(OH)CHCH O 3 2 12 2 NHAcyl CH CH N(CH ) 2 2 33 + O- 3.5.1.23 1.13.11.21 2.5.1.32 1.1.1.105 2.4.2.19 1.2.1.32 METHIONINE 4.1.3.27 4.2.1.51 1.14.17.1 1.14.18.1 1.14.13.11 Glycinamide- ribosyl-P 4.2.1.22 3-Oxohexanoyl-CoA THREONINE 1.5.1.7 - 10 GLUTAMATE 2.3.1.38 ATP NADH SUCROSE ADENOSINE-P (AMP) Triacylglycerol FAT RNA CH 3 CH 2 OC-COO OH COO CH CH 2 COO (NH ) 3 + GLYCOPROTEINS GANGLIOSIDES MUCINS 4.1.2.- 2.6.1.- GLUCOSE O-Acyl-carnitine O-Acyl-carnitine OH O - - R-CO-OCH R'-CO-OCH R'-CO-OCH O HCO-CO-R CH CH(OH)CH O-P-OCH 2 2 2 CH O-CO-R’ 2 O R'-CO-OCH O- CH O-CO-R 2 CH O-PO 2 O- 2.7.8.11 H NCH CH CH CH 2 2 2 2 COO (NH ) 3 + 2.3.1.20 3.1.3.4 2.7.1.107 NH 2 + + H NCNHCH CH CH CH 2 2 2 2 COO (NH ) 3 H OH H OH C C C CO OH H HOCH2 CH OH 2 R-CH 2 COO CH O-CO-R 2 CH O-CO-R" 2 R’-CO-OCH OH O CH CHNH 2 3 + O R'-CO-OCH O- CH O-CO-R 2 CH O PO 2 COO OOCCH CH CH 2 2 COO (NH ) 3 + CH CH(OH)CH(NH )COO 3 3 O OH HO OH OH CH OH 2 CH OH 2 NH CH O-CO-R 2 1.3.1.13 4.1.1.28 Malonic semi- aldehyde 4 . 2 . 7 . 2 2.1.3.3 3.5.1.2 6.3.1.2 5 . 5 . 3 . 6 Carbohydrates Biosynthesis Degradation Amino Acids Biosynthesis Degradation Lipids Biosynthesis Degradation Purines & Pyrimidines Biosynthesis Degradation Biosynthesis Degradation Vitamins Co-enzymes & Hormones Pentose Phosphate Pathway Photosynthesis Dark Reactions COMPARTMENTATION The "Backbone" of metabolism involves GLYCOLYSIS in the CYTOPLASM, the TCA CYCLE (mainly) in the Mitochondrial matrix and ATP FORMATION spanning the MITOCHONDRIAL INNER MEMBRANE An electron flow (an electric current) generated from NADH and UQH2 drives the translocation of protons from the matrix to the intermembrane space. The retrolocation of these protons through the F0 subunits of ATP synthase to the matrix then supplies the energy needed to form ATP from ADP and phosphate Electron Flow Proton Flow Small Numbers ( eg. 2.4.6.7) refer to the IUBMBEnzyme Commission (EC) Reference Numbers of Enzymes LEGEND Human Metabolism is identified as far possible by black arrows Biosynthesis Degradation HEMOGLOBIN CHLOROPHYLL ATP T R A N S L O C A T E D PROTONS 2.7.1.1 ATP 2.7.1.2 3.7.1.2 2.4.1.1 3.2.1.33 3.2.1.23 3.2.1.108 2.7.1.6 2.7.7.12 3.1.3.9 3.2.1.10 2.4.1.25 2.4.2.8 3.5.4.4 1.5.1.3 1.4.4.2 2.6.1.44 4.1.3.4 1.9.3.1 4.3.1.8 4.1.1.37 1.3.3.4 1.3.99.3 3.2.1.45 3.2.1.52 3.1.4.12 1.14.16.1 1.14.18.1 1.17.3.2 4.2.1.22 6.4.1.3 1.2.1.24 2.6.1.19 6.3.4.16 2.1.3.3 4.3.2.1 6.3.4.5 5.4.99.2 1.3.99.10 1.2.4.4 1.2.4.4 1.2.4.4 © 2006 International Union of Biochemistry and Molecular Biology www.iubmb.org Designed by Donald E.Nicholson,The University of Leeds,England – and Sigma-Aldrich in collaboration with Mick Henderson,St.James’ Hospital,Leeds – and the UK MetBioNet Training Group www/metbio.net. For further information about metabolic disorders visit the OMIM (Online Mendelian Inheritance in Main) website, hosted by the J ohn Hopkins University – www.ncbi.nlm.gov/entrez/query/fcgi?db=OMIM. GDS Inborn Errors of Metabolism AMINO ACID METABOLISM Fumarylacetoacetase (3.7.1.2) Tyrosinaemia Type I MIM 276700 Phenylalanine hydroxylase (1.14.16.1) Phenylketonuria (PKU) MIM 261600 Ty rosinase (1.14.18.1) Tyrosine Negative Albinism MIM 203100 Glycine dehydrogenase (1.4.4.2) Non Ketotic Hyperglycinaemia (NKH) MIM 238300 PURINE & PYRIMIDINE METABOLISM Xanthine oxidase (1.17.3.2) Xanthinuria MIM 278300 Hypoxanthine guanine phospho-ribosyl transferase (2.4.2.8) HGPRT Deficiencey, Primary Gout, Lesch-Nyhan Syndrome MIM 308000 Adenosine deaminase (3.5.4.4) Severe Combined Immuno- Deficiency (SCID) MIM 102700 FOLIC ACID Dihydrofolate reductase (1.5.1.3) Megaloblastic Anaemia MIM 126060 ORGANIC ACIDURIAS Propionyl-CoA carboxylase (6.4.1.3) Propionic Acidaemia MIM 606054 Methylmalonyl-CoA mutase (5.4.99.2) Methylmalonic Acidaemia MIM 251000 Isovaleryl-CoA dehydrogenase (1.3.99.10) Isovaleric Acidaemia MIM 243500 AMINO ACID METABOLISM Cystathionine synthetase (4.2.1.22) Homocystinuria MIM 236200 Branched chain ketoacid decarboxylase (1.2.4.4) Maple Syrup Urine Disease MIM 248600 GABA METABOLISM NEUROTRANSMITTER DEFECTS Succinic semialdehyde dehydrogenase (1.2.1.24) 4-Hydroxybutyric Aciduria MIM 271980 4-Aminobutyrate aminotransferase (2.6.1.19) GABA Transferase Deficiency MIM 137150 UREA CYCLE DEFECTS (HYPERAMMONAEMIA) Carbamoyl phosphate synthase (6.3.4.16) CPS Deficiency MIM 237300 Ornithine carbamoyl transferase (2.1.3.3) OCT Deficiency MIM 300161 Argininosuccinate synthase (6.3.4.5) Citrullinaemia MIM 215700 Argininosuccinate lyase (4.3.2.1) Argininosuccinate Aciduria MIM 207900 GLYCOGEN STORAGE DISEASES MUSCLE GLYCOGENOSES Muscle glycogen phosphorylase (2.4.1.1) GSD Type V McArdle Disease MIM 232600 LIVER GLYCOGENOSES Amylo-1,6-glucosidase (3.2.1.33) Glucanotransferase (2.4.1.25) GSD Type III Cori Disease MIM 232400 Glucose-6-phosphatase (3.1.3.9) GSD Type I Von Gierke Disease MIM 232200 MONO- & DI-SACCHARIDES Sucrase-isomaltase (3.2.1.10) Sucrose-Isomaltose Malabsorption MIM 609845 Lactase (3.2.1.23) (3.2.1.108) Lactose Intolerance MIM 223000 Galactokinase (2.7.1.6) Galactose Kinase Deficiency MIM 230200 Galactose-1-phosphate uridyl transferase (2.7.7.12) Classical Galactosaemia MIM 606999 PEROXISOMAL DEFECTS Alanine-glyoxylate aminotransferase (2.6.1.44) Hyperoxaluria Type 1 MIM 259900 LIPID METABOLISM Hydroxymethylglutaryl CoA lyase (4.1.3.4) Ketone Synthesis Defect MIM 246450 Medium chain acyl CoA dehydrogenase (1.3.99.3) MCAD Deficiency MIM 607008 SPHINGOLIPIDOSES Acid-ß-glucosidase (3.2.1.45) Gaucher Disease MIM 230800 Hexosaminidase A (3.2.1.52) Tay Sachs Disease MIM 272800 Sphingomyelinase (3.1.4.12) Niemann-Pick Disease MIM 257200 RESPIRATORY CHAIN DEFECTS Cytochrome c oxidase (1.9.3.1) Complex IV MIM 220110 PORPHYRIAS Porphobilinogen deaminase (2.5.1.61) Acute Intermittent Porphyria MIM 176000 Uroporphyrinogen decarboxylase (4.1.1.37) Porphyria Cutanea Tarda MIM 176100 Protoporphyrinogen oxidase (1.3.3.4) Variegate Porphyria MIM 176200 sigma-aldrich.com/pathways