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ENZYME entry: EC

Accepted Name
Branched-chain alpha-keto acid dehydrogenase system.
Alternative Name(s)
2-oxoisovalerate dehydrogenase (acylating).
Branched-chain alpha-keto acid dehydrogenase complex.
Reaction catalysed
3-methyl-2-oxobutanoate + CoA + NAD(+) <=> 2-methylpropanoyl-CoA + CO(2) + NADH
  • This enzyme system catalyzes the oxidative decarboxylation of branched-chain alpha-keto acids derived from L-leucine, L-isoleucine, and L-valine to branched-chain acyl-CoAs.
  • It belongs to the 2-oxo acid dehydrogenase system family, which also includes the pyruvate dehydrogenase system, 2-oxoglutarate dehydrogenase system, and glycine cleavage system.
  • With the exception of the glycine cleavage system, the 2-oxo acid dehydrogenase systems share a common structure, consisting of three main components, namely a 2-oxo acid dehydrogenase (E1), a dihydrolipoamide acyltransferase (E2), and dihydrolipoamide dehydrogenase (E3).
  • The reaction catalyzed by this system is the sum of three activities: EC, EC and EC
  • The system also acts on (S)-3-methyl-2-oxopentanoate and 4-methyl-2- oxopentanoate.
PRIAM enzyme-specific profiles1.2.1.25
KEGG Ligand Database for Enzyme Nomenclature1.2.1.25
IUBMB Enzyme Nomenclature1.2.1.25
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