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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.
AA959893; C10orf7; Cdc123; cell division cycle 123; cell division cycle 123 homolog; cell division cycle protein 123 homolog; Cell division cycle protein 123-like protein; D123; D123 gene product; G431001I09Rik; HT-1080; hypothetical protein; Protein D123; PZ32; QccE-12339
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