Tags: Protein.
2-oxoisovalerate dehydrogenase subunit alpha mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene.The second major step in the catabolism of the branched-chain amino acids isoleucine leucine and valine is catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD; EC 1.2.4.4) an inner-mitochondrial enzyme complex that consists of 3 catalytic components: a heterotetrameric (alpha2 beta2) branched-chain alpha-keto acid decarboxylase (E1) a homo-24-meric dihydrolipoyl transacylase (E2; MIM 248610) and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331).