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Summary of branched chain amino acid metabolism. Enzymes involved in the catabolism of leucine, isoleucine, and valine are shown in blue boxes.
Disorders discussed in this chapter are numbered and start at the level of the branched chain acyl-CoA dehydrogenases (1, isovaleryl-CoA
dehydrogenase; 2, short/branched chain acyl-CoA dehydrogenase; and 3, isobutyryl-CoA dehydrogenase). The remaining enzymes in the pathway are 4,
3-methylcrotanyl-CoA carboxylase; 5, tiglyl-CoA hydratase; 6, methacrylyl-CoA hydratase; 7, 3-methylglutaconyl-CoA hydratase; 8, 2-mthyl-3hydroxybutyryl-CoA dehydrogenase; 9, 3-hydroxyisobutyryl-CoA deacylase; 10, 3-hydroxy-3-methylglutaryl-CoA lyase; 11, 2-methyl-acetoacetyl-CoA
thiolase; 12, 3-hydroxyisobutyryl-CoA dehydrogenase; 13, methylmalonate semialdehyde dehydrogenase. The aminotransferases, branched chain
Source: Branched Chain Organic Acidurias, The Online Metabolic and Molecular Bases of Inherited Disease
ketoacid dehydrogenase, propionyl-CoA carboxylase, and methylmalonyl-CoA mutase are discussed in other chapters.
Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular
Bases of Inherited Disease; 2014 Available at: http://mhmedical.com/ Accessed: May 12, 2017
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