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ENZYME

ENZYME entry: EC 1.2.4.4

PURL: https://purl.expasy.org/enzyme/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
N(6)-[(R)-lipoyl]-L-lysyl-[protein] + 3-methyl-2-oxobutanoate + H(+) = N(6)-[(R)-S(8)-2-methylpropanoyldihydrolipoyl]-L-lysyl-[protein] + CO2
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
BRENDA1.2.4.4
EC2PDB1.2.4.4
ExplorEnz1.2.4.4
KEGG Ligand Database for Enzyme Nomenclature1.2.4.4
IUBMB Enzyme Nomenclature1.2.4.4
MEDLINEFind literature relating to 1.2.4.4
MetaCyc1.2.4.4
Rhea expert-curated reactions1.2.4.4
UniProtKB/Swiss-Prot
P9WIS2, BKDA_MYCTOP9WIS3, BKDA_MYCTUP9WIS0, BKDB_MYCTO
P9WIS1, BKDB_MYCTUQ9LPL5, ODBA1_ARATHQ84JL2, ODBA2_ARATH
P37940, ODBA_BACSUP11178, ODBA_BOVINO45924, ODBA_CAEEL
Q54M22, ODBA_DICDIP12694, ODBA_HUMANQ8HXY4, ODBA_MACFA
P50136, ODBA_MOUSEA5A6H9, ODBA_PANTRQ9I1M2, ODBA_PSEAE
P09060, ODBA_PSEPUP11960, ODBA_RATQ72GU1, ODBA_THET2
Q5SLR4, ODBA_THET8Q9SAV3, ODBB1_ARATHQ9LDY2, ODBB2_ARATH
P37941, ODBB_BACSUP21839, ODBB_BOVINP9WF02, ODBB_CHATD
Q55FN7, ODBB_DICDIP21953, ODBB_HUMANQ6P3A8, ODBB_MOUSE
Q9I1M1, ODBB_PSEAEP09061, ODBB_PSEPUP35738, ODBB_RAT
Q72GU2, ODBB_THET2Q5SLR3, ODBB_THET8

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