BCKDE1A Antibody

BCKDE1A (branched chain keto acid dehydrogenase E1, alpha polypeptide) is a component of the branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex. The BCKD complex is an inner mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). Mutations in this gene result in maple syrup urine disease, type IA [taken from NCBI Entrez Gene (Gene ID: 593)].

Alternative names for BCKDE1A Antibody include 2-oxoisovalerate dehydrogenase subunit alpha mitochondrial antibody, branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, BCKDE1A antibody, BCKDH E1-alpha antibody, BCKDHA antibody, MSU antibody, MSUD1 antibody, OVD1A antibody, FLJ45695 antibody.