Normal IVA Protein from food Protein from muscles Amino Acids Energy Amino Acids Isovaleryl CoA dehydrogenase enzyme Build up of Isovaleric acid + other harmful substances Health Problems Protein from muscles Protein from food Leucine Isovaleryl CoA dehydrogenase enzyme Isovaleric acid Growth Other amino acids Energy Growth Other amino acids Leucine Isovaleric acid Isovaleric Acidemia (IVA)