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Metabolic pathways - catabolism of branched-chain amino acids (2)

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

Associated Disorders

  • Hyperreflexia
  • Infantile spasms
  • Mental retardation
  • Muscular hypotonia
  • Neutrophilia
  • Reye syndrome
  • Seizures