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Information on EC - branched-chain alpha-keto acid dehydrogenase system

for references in articles please use BRENDA:EC1.2.1.25
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IUBMB Comments
This enzyme system catalyses 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 catalysed by this system is the sum of three activities: EC, 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring), EC, dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, and EC, dihydrolipoyl dehydrogenase. The system also acts on (S)-3-methyl-2-oxopentanoate and 4-methyl-2-oxopentanoate.
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The enzyme appears in viruses and cellular organisms
2-oxoisovalerate dehydrogenase, alpha-ketoisovalerate dehydrogenase, dehydrogenase, 2-oxoisovalerate (acylating), more
3-methyl-2-oxobutanoate + CoA + NAD+ = 2-methylpropanoyl-CoA + CO2 + NADH
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