BCKDHA Recombinant Rabbit Monoclonal Antibody - BSA and Azide free [PSH15-62] (HA751573)

UNIPROT #

P12694

PROTEIN NAME

BCKDHA

SYNONYMS

Branched chain alpha keto acid dehydrogenase E1 component alpha polypeptide antibody;FLJ45695 antibody;OVD1A antibody;2 oxoisovalerate dehydrogenase (lipoamide) antibody;2 oxoisovalerate dehydrogenase subunit alpha, mitochondrial antibody;2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial antibody;BCKDE1A antibody;BCKDH E1 alpha antibody;BCKDH E1-alpha antibody;BCKDHA antibody;Branched chain alpha keto acid dehydrogenase E1 component alpha chain antibody;Branched chain keto acid dehydrogenase E1 alpha polypeptide antibody;Branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) antibody;Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain antibody;MSU antibody;MSUD1 antibody;ODBA_HUMAN antibody

SUBCELLULAR LOCATION

Mitochondrion matrix.

FUNCTION

A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene. BCKDHA is a coding gene that is part of the BCKD complex (branched-chain alpha-keto acid dehydrogenase). 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). The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase. The BCKDHA gene encodes the alpha subunit of E1, and the BCKDHB gene (MIM 248611) encodes the beta subunit of E1.