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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)

Expression

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

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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
donor hepatocytes expressing wild type BCKDH engrafted into intermediate maple syrup urine disease recipient liver, increase liver BCKDH activity. Hepatocyte transplantation increases liver BCKDH activity 14.36% of control activity
-
in skeletal muscle, subunits E1alpha protein is significantly reduced in OLETF rats
-
mRNA expression is not upregulated by bezafibrate treatment
-
the total amount of hepatic enzyme is significantly decreased in diet-induced obesity rats
-
total BCKDC activity and protein abundance of E1alpha, E1beta, and E2 subunits are markedly lower in Otsuka Long-Evans Tokushima Fatty (OLETF) than in Long-Evans Tokushima Otsuka (LETO) rats at 8 and 25 weeks of age. In addition, hepatic BCKDC activity and protein amounts are significantly decreased in LETO rats aged 25 weeks than in LETO rats aged 8 weeks. In skeletal muscle, subunits E1beta and E2 proteins are significantly reduced in OLETF rats
-