BCKDHA

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BCKDHA
Protein BCKDHA PDB 1dtw.png
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases BCKDHA, BCKDE1A, MSU, MSUD1, OVD1A, branched chain keto acid dehydrogenase E1, alpha polypeptide
External IDs MGI: 107701 HomoloGene: 569 GeneCards: BCKDHA
Orthologs
Species Human Mouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_001164783
NM_000709

NM_007533

RefSeq (protein)

NP_000700
NP_001158255

n/a

Location (UCSC) Chr 19: 41.4 – 41.43 Mb Chr 7: 25.63 – 25.66 Mb
PubMed search [1] [2]
Wikidata
View/Edit Human View/Edit Mouse

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene.[3]

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.[supplied by OMIM][3]

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