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ONTOLOGY REPORT - ANNOTATIONS

Term:branched-chain alpha-ketoacid dehydrogenase complex
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Accession:GO:0160157 term browser browse the term
Definition:A multi-enzyme complex that catalyzes the oxidative decarboxylation of branched-chain alpha-ketoacids derived from L-leucine, L-isoleucine, and L-valine to branched-chain acyl-CoAs. The complex comprises multiple copies of three enzymes referred to as E1, E2 and E3: branched-chain alpha-ketoacid dehydrogenase (E1, a heterotetramer of two alpha and two beta subunits), dihydrolipoyl transacylase (E2), and dihydrolipoamide dehydrogenase (E3). Additional proteins may also be present.
Comment:The catalytic activities of the individual components of this complex are represented by the molecular function terms '3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity ; GO:0003863' (E1), 'dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity ; GO:0043754' (E2), and 'dihydrolipoyl dehydrogenase activity ; GO:0004148' (E3). The overall catalytic activity of the complex is represented by the molecular function term 'branched-chain alpha-keto acid dehydrogenase activity (GO:0047101)'.
Synonyms:exact_synonym: BCKD complex;   BKDH complex
 xref: Wikipedia:Branched-chain_alpha-keto_acid_dehydrogenase_complex


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branched-chain alpha-ketoacid dehydrogenase complex term browser
Symbol Object Name Qualifiers Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G BCKDHA branched chain keto acid dehydrogenase E1 subunit alpha part_of ISO (PMID:10745006) UniProt PMID:10745006 NCBI chr 1:112,579,345...112,599,163 JBrowse link
G BCKDHB branched chain keto acid dehydrogenase E1 subunit beta part_of ISO (PMID:10745006) UniProt PMID:10745006 NCBI chr12:41,009,798...41,224,802
Ensembl chr12:41,009,730...41,224,802 		JBrowse link
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  cellular_component 18906
    protein-containing complex 6503
      catalytic complex 1856
        oxidoreductase complex 166
          alpha-ketoacid dehydrogenase complex 19
            branched-chain alpha-ketoacid dehydrogenase complex 2
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