3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) EC#: 1.2.4.4; ChemWhat Code: 1373896

Product Name 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)
Example Structure Example Structure of 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) EC#: 1.2.4.4
Synonyms 2-oxoisocaproate dehydrogenase, 2-oxoisovalerate dehydrogenase, 2-oxoisovalerate dehydrogenase (lipoate), alpha-keto-beta-methylvalerate dehydrogenase, alpha-ketoacid dehydrogenase, alpha-ketoisocaproate dehydrogenase, alpha-ketoisocaproic dehydrogenase, alpha-ketoisocaproic-alpha-keto-beta-methylvaleric dehydrogenase, alpha-ketoisovalerate dehydrogenase, alpha-oxoisocaproate dehydrogenase, BCDH, BCKA dehydrogenase, BCKADC, BCKD, BCKDC, BCKDH, BCKDH E1-alpha, BCKDH E1-beta, BCKDHB, BCKDHc, BCOA, BCOAD, Bkd, bkdF, branched chain alpha-ketoacid dehydrogenase complex, branched chain keto acid dehydrogenase, branched-chain (-2-oxoacid BCD) dehydrogenase, branched-chain 2-keto acid dehydrogenase, branched-chain 2-oxo acid dehydrogenase, branched-chain alpha-keto acid decarboxylase/dehydrogenase, branched-chain alpha-keto acid dehydrogenase, branched-chain alpha-keto acid dehydrogenase complex, branched-chain alpha-keto acid dehydrogenase E1beta subunit, branched-chain alpha-ketoacid dehydrogenase, branched-chain alpha-ketoacid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase enzyme complex, branched-chain alpha-ketoacid dehydrogenase multienzyme complex, branched-chain alpha-oxo acid dehydrogenase, branched-chain keto acid dehydrogenase, branched-chain keto acid dehydrogenase complex, branched-chain ketoacid dehydrogenase, branched-chain ketoacid dehydrogenase E1, dehydrogenase, 2-oxoisocaproate, dehydrogenase, 2-oxoisovalerate (lipoate), dehydrogenase, branched chain alpha-keto acid, DHalpha, dihydrolipoyl acyl-transferase, dihydrolipoyl transacylase, E1b, E1b component, E1b component of the 2-oxo acid dehydrogenase complex, E2, EC 1.2.4.3, More
EC Number 1.2.4.4
CAS Registry Number 9082-72-8
Comments Contains thiamine diphosphate. It acts not only on 3-methyl-2-oxobutanaoate, but also on 4-methyl-2-oxopentanoate and (S)-3-methyl-2-oxopentanoate, so that it acts on the 2-oxo acids that derive from the action of transaminases on valine, leucine and isoleucine. It is a component of the multienzyme 3-methyl-2-oxobutanoate dehydrogenase complex in which multiple copies of it are bound to a core of molecules of EC 2.3.1.168, dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, which also binds multiple copies of EC 1.8.1.4, dihydrolipoyl dehydrogenase. It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.168.
Cofactor thiamine diphosphate
History
Reactions 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine- residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO(2).

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