1.2.1.25: branched-chain alpha-keto acid dehydrogenase system

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For detailed information about branched-chain alpha-keto acid dehydrogenase system, go to the full flat file.

Reaction

Synonyms

2-oxoisovalerate dehydrogenase, alpha-ketoisovalerate dehydrogenase, BCKD, BCKD complex, BCKDalpha, BCKDbeta, BCKDC, BCKDH, BCKDHA, BCKDHB, bkdA, BKDH, branched chain alpha-keto acid dehydrogenase complex, branched chain keto-acid dehydrogenase, branched-chain alpha-keto acid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase, branched-chain alpha-ketoacid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase complex E1alpha subunit, branched-chain ketoacid dehydrogenase, branched-chain-keto-acid dehydrogenase alpha, branched-chain-keto-acid dehydrogenase beta, dehydrogenase, 2-oxoisovalerate (acylating), E1alpha subunit, E1beta subunit, mitochondrial branched-chain keto-acid dehydrogenase, More

ECTree

     1 Oxidoreductases

         1.2 Acting on the aldehyde or oxo group of donors

             1.2.1 With NAD⁺ or NADP⁺ as acceptor

                1.2.1.25 branched-chain alpha-keto acid dehydrogenase system

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3	Cloned(Commentary)
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114	Disease/ Diagnostics
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35	General Information
7	Inhibitors
2	KM Value [mM]
11	Localization
38	Natural Substrates/ Products (Substrates)
188	Organism
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47	Synonyms
1	Systematic Name

Systematic Name

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Systematic Name on EC 1.2.1.25 - branched-chain alpha-keto acid dehydrogenase system

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SYSTEMATIC NAME

IUBMB Comments

3-methyl-2-oxobutanoate:NAD+ 2-oxidoreductase (CoA-methylpropanoylating)

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-oxoacid dehydrogenase system family, which also includes EC 1.2.1.104, pyruvate dehydrogenase system, EC 1.2.1.105, 2-oxoglutarate dehydrogenase system, EC 1.4.1.27, glycine cleavage system, and EC 2.3.1.190, acetoin dehydrogenase system. With the exception of the glycine cleavage system, which contains 4 components, the 2-oxoacid dehydrogenase systems share a common structure, consisting of three main components, namely a 2-oxoacid dehydrogenase (E1), a dihydrolipoamide acyltransferase (E2), and dihydrolipoamide dehydrogenase (E3). The reaction catalysed by this system is the sum of three activities: EC 1.2.4.4, 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring), EC 2.3.1.168, dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, and EC 1.8.1.4, dihydrolipoyl dehydrogenase. The system also acts on (S)-3-methyl-2-oxopentanoate and 4-methyl-2-oxopentanoate.