Sign Up for a Free Account

Metabolic pathways: catabolism of branched-chain amino acids (1)

ICD = isovaleryl-CoA dehydrogenase, deficient in isovaleric acidemia, resulting in increased concentrations of hydroxyisovaleric acid and other metabolites; BCKDH = branched chain alpha-keto acid dehydrogenase, deficient in MSUD; MCC = 3-methylcrotonyl-CoA carboxylase, deficient in methylcrotonylglycinuria; DH = dehydrogenase; PCC = propionyl-CoA carboxylase, deficient in propionic acidemia; MCM = methylmalonyl CoA mutase, deficient in methylmalonic aciduria. (Contributed by Dr. Georg F Hoffmann.)