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ENZYME

ENZYME entry: EC 1.2.4.4

Accepted Name
3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)
Alternative Name(s)
2-oxoisocaproate dehydrogenase
2-oxoisovalerate (lipoate) dehydrogenase
3-methyl-2-oxobutanoate dehydrogenase (lipoamide)
alpha-keto-alpha-methylvalerate dehydrogenase
alpha-ketoisocaproate dehydrogenase
alpha-ketoisocaproic-alpha-keto-alpha-methylvaleric dehydrogenase
alpha-ketoisocaproic dehydrogenase
alpha-ketoisovalerate dehydrogenase
alpha-oxoisocaproate dehydrogenase
BCKDH
BCOAD
branched-chain (-2-oxoacid) dehydrogenase (BCD)
branched-chain 2-keto acid dehydrogenase
branched-chain 2-oxo acid dehydrogenase
branched-chain alpha-keto acid dehydrogenase
branched-chain alpha-oxo acid dehydrogenase
branched chain keto acid dehydrogenase
branched-chain ketoacid dehydrogenase
branched-chain keto acid dehydrogenase
dehydrogenase, 2-oxoisovalerate (lipoate)
Reaction catalysed
3-methyl-2-oxobutanoate + H(+) + N(6)-[(R)-lipoyl]-L-lysyl-[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] <=> CO2 + N(6)-[(R)-S(8)-2-methylpropanoyldihydrolipoyl]-L-lysyl-[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
Comment(s)
  • 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, which also binds multiple copies of EC 1.8.1.4.
  • It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.168.
  • Formerly EC 1.2.4.3.
Cross-references
BRENDA 1.2.4.4
EC2PDB 1.2.4.4
ExplorEnz 1.2.4.4
PRIAM enzyme-specific profiles 1.2.4.4
KEGG Ligand Database for Enzyme Nomenclature 1.2.4.4
IUBMB Enzyme Nomenclature 1.2.4.4
IntEnz 1.2.4.4
MEDLINE Find literature relating to 1.2.4.4
MetaCyc 1.2.4.4
Rhea expert-curated reactions 1.2.4.4
UniProtKB/Swiss-Prot
P9WIS2, BKDA_MYCTO P9WIS3, BKDA_MYCTU P9WIS0, BKDB_MYCTO
P9WIS1, BKDB_MYCTU Q9LPL5, ODBA1_ARATH Q84JL2, ODBA2_ARATH
P37940, ODBA_BACSU P11178, ODBA_BOVIN O45924, ODBA_CAEEL
Q54M22, ODBA_DICDI P12694, ODBA_HUMAN Q8HXY4, ODBA_MACFA
P50136, ODBA_MOUSE A5A6H9, ODBA_PANTR Q9I1M2, ODBA_PSEAE
P09060, ODBA_PSEPU P11960, ODBA_RAT Q72GU1, ODBA_THET2
Q5SLR4, ODBA_THET8 Q9SAV3, ODBB1_ARATH Q9LDY2, ODBB2_ARATH
P37941, ODBB_BACSU P21839, ODBB_BOVIN P9WF02, ODBB_CHATD
Q55FN7, ODBB_DICDI P21953, ODBB_HUMAN Q6P3A8, ODBB_MOUSE
Q9I1M1, ODBB_PSEAE P09061, ODBB_PSEPU P35738, ODBB_RAT
Q72GU2, ODBB_THET2 Q5SLR3, ODBB_THET8

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All ENZYME / UniProtKB/Swiss-Prot entries corresponding to 1.2.4.-
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