BCKDHA Products

Description

The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine. This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998 [PubMed 9582350]). The BCKD complex 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). E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene (MIM 248611) encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.[supplied by OMIM]

Bioinformatics

Entrez	593 Human
Uniprot	NP_000700.1 Human
Product By Gene ID	593
Alternate Names	2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial BCKDE1A BCKDH E1-alpha branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urinedisease) branched chain keto acid dehydrogenase E1, alpha polypeptide Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain EC 1.2.4.4,2-oxoisovalerate dehydrogenase (lipoamide) FLJ45695 MSU MSUD1 OVD1A

Research Areas for BCKDHA

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Phospho-Specific