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EC Number
Natural Substrates
Commentary (Nat. Sub.)
1.2.1.25
3-methyl-2-oxobutanoate + CoA + NAD+
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1.2.1.25
3-methyl-2-oxopentanoate + CoA + NAD+
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1.2.1.25
4-methyl-2-oxopentanoate + CoA + NAD+
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1.2.1.25
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the human BCKD complex catalyzes the irreversible oxidative decarboxylation of the branched-chain ketoacids (2-keto-isocaproate derived from leucine, 2-keto-3-methylglutarate obtained from isoleucine, and 2-keto-isovalerate from valine). This reaction produces CO2, NADH, and the respective branched-chain acyl-coA intermediates with a 1:1:1 stoichiometry. The action of BCKD complex generates isovaleryl-CoA from 2-ketoisocaproate (leucine), 2-methylbutyryl-CoA from 2-keto-3-methylglutarate (isoleucine), and isobutyryl-CoA from 2-keto-isovalerate (valine)
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