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Metabolic role and context of branched-chain keto acid dehydrogenase complex

BCAA = Branched-Chain Amino Acids; BCKA = Branched-Chain Keto Acids; BC acyl-CoAs = Branched-Chain acyl-CoAs; BCATm = mitochondrial branched-chain aminotransferase; BCATc = cytoplasmic branched-chain aminotransferase; KMV = alpha-keto-beta-methylvalerate; KIC = alpha-ketoisocaproate; KIV = alpha-ketoisovalerate. (Contributed by Dr. Brendan Lee.)

Associated Disorders

  • Disorder of amino acid metabolism
  • Inherited metabolic disorder
  • Organic aciduria