Branched-chain alpha-keto acid dehydrogenase complex

The branched-chain α-ketoacid dehydrogenase complex (BCKDC or BCKDH complex) is a multi-subunit complex of enzymes that is found on the mitochondrial inner membrane.[1] This enzyme complex catalyzes the oxidative decarboxylation of branched, short-chain alpha-ketoacids. BCKDC is a member of the mitochondrial α-ketoacid dehydrogenase complex family, which also includes pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase, key enzymes that function in the Krebs cycle.

  1. ^ Indo I, Kitano A, Endo F, Akaboshi I, Matsuda I (1987). "Altered Kinetic Properties of the Branched-Chain Alpha-Keto Acid Dehydrogenase Complex Due to Mutation of the Beta-Subunit of the Branched-Chain Alpha-Keto Acid Decarboxylase (E1) Component in Lymphoblastoid Cells Derived from Patients with Maple Syrup Urine Disease". J Clin Invest. 80 (1): 63–70. doi:10.1172/JCI113064. PMC 442202. PMID 3597778.