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BCKDHA

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Identifiers
SymbolsBCKDHA ; BCKDE1A; MSU; MSUD1; OVD1A
External IDsOMIM608348 MGI107701 HomoloGene569 GeneCards: BCKDHA Gene
EC number1.2.4.4
Orthologs
SpeciesHumanMouse
Entrez59312039
EnsemblENSG00000248098ENSMUSG00000060376
UniProtP12694Q3U3J1
RefSeq (mRNA)NM_000709NM_007533
RefSeq (protein)NP_000700NP_031559
Location (UCSC)Chr 19:
41.88 – 41.93 Mb
Chr 7:
25.63 – 25.66 Mb
PubMed search[1][2]

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene.[1]

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][1]

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