The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid. 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, and a homodimeric dihydrolipoamide dehydrogenase. E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: cell division cycle 123 homolog; Cell division cycle protein 123 homolog; HT-1080; Protein D123; PZ32
Gene Aliases: C10orf7; CDC123; D123
UniProt ID: (Human) O75794