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1.2.4.4: 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

This is an abbreviated version!
For detailed information about 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring), go to the full flat file.

Word Map on EC 1.2.4.4

Reaction

3-methyl-2-oxobutanoate
+
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine
=
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine
+
CO2
+ 2 H+

Synonyms

2-oxoisocaproate dehydrogenase, 2-oxoisovalerate dehydrogenase, 2-oxoisovalerate dehydrogenase (lipoate), 2-oxoisovalerate dehydrogenase subunit alpha, 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, BCKDHA, BCKDHB, BCKDHc, BCOA, BCOAD, BDKG, 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

ECTree

     1 Oxidoreductases
         1.2 Acting on the aldehyde or oxo group of donors
             1.2.4 With a disulfide as acceptor
                1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

Inhibitors

Inhibitors on EC 1.2.4.4 - 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
(S)-2-chloro-phenylpropionic acid
-
-
2-chloro-4-methylpentanoate
-
inhibits component E1, modifies a histidine side chain
2-chloroisocaproate
-
-
2-oxobutanoate
-
-
2-oxoglutarate
-
-
2-oxopentanoate
-
-
3-methyl-2-oxobutanoate
-
substrate inhibition
3-methyl-2-oxopentanoate
-
-
3-methylbutanoyl-CoA
4-(2-Thienyl)-2-oxo-3-butenoate
-
-
4-(3-Thienyl)-2-oxo-3-butenoate
4-methyl-2-oxopentanoate
alpha-Ketoisocaproate
-
allosteric inhibition, also by other branched-chain keto acids, although they are less effective as compared to alpha-ketoisocaproate
cinnamylpyruvate
Clofibrate
-
-
D-3-methyl-2-oxopentanoate
-
substrate inhibition
Furfurylidenepyruvate
isobutanoyl-CoA
-
0.2 mM, 48% inhibition
isopentanoyl-CoA
L-3-Methyl-2-oxopentanoate
-
substrate inhibition
microRNA 29a
-
total BCKD activity decreases over a 6-day period following a single transfection
-
microRNA 29b
-
total BCKD activity decreases over a 6-day period following a single transfection, is targeted to the mRNA for the dihydrolipoamide branched chain acyltransferase component of BCKD and prevents translation when bound
-
Skeletal muscle factor
-
activity from liver, kidney and brains
-
additional information
-