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
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1.2.4.4
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urine
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maple
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syrup
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valine
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isoleucine
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ketoacids
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thiamin
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dihydrolipoamide
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aminotransferase
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bckas
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alpha-ketoglutarate
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e1alpha
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transamination
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lipoic
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lipoamide
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acidosis
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lipoylated
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2-oxoglutarate
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ketoacidosis
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bcatm
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l-leucine
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mennonite
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acidemia
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low-protein
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isovaleryl-coa
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clofibric
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alpha2beta2
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antimitochondrial
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dichloroacetate
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groes
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2-oxoacids
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synthesis
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medicine
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1-14c-labelled
- 1.2.4.4
- urine
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maple
- syrup
- valine
- isoleucine
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ketoacids
- thiamin
- dihydrolipoamide
- aminotransferase
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bckas
- alpha-ketoglutarate
- e1alpha
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transamination
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lipoic
- lipoamide
- acidosis
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lipoylated
- 2-oxoglutarate
- ketoacidosis
- bcatm
- l-leucine
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mennonite
- acidemia
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low-protein
- isovaleryl-coa
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clofibric
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alpha2beta2
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antimitochondrial
- dichloroacetate
- groes
- 2-oxoacids
- synthesis
- medicine
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1-14c-labelled
Reaction
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
1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)Advanced search results
results (
results (Activating Compound
Activating Compound on EC 1.2.4.4 - 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)
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ACTIVATING COMPOUND 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
IMAGE
5-fluorouracil
in male rats administered with 4 doses of 5-FU, 150 mg/kg b.wt. each, 5-FU treatment causes an increase in branched-chain alpha-keto acid dehydrogenase complex BCKDH activity that results mainly from increased dephosphorylation of the complex and is associated with an increase of phosphatase PPM1K mRNA level and reduction of BDK and dehydrogenase E1 mRNA levels
Activator protein
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a protein in rat liver and kidney mitochondria that reactivates phosphorylated branched-chain complex without dephosphorylation
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alpha-ketoisovalerate
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activates through release of branched-chain alpha-keto acid dehydrogenase kinase from the BCKD complex
bezafibrate
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i.e. 2-[4-[2-(4-chlorobenzamido)ethyl]phenoxy]-2-methylpropanoic acid. In rats treated with 5, 10, 20 mg/kg bezafibrate mean enzyme actual activity is 1.9, 2.9, and 4.3fold higher than in the control group, respectively. Enzymatic total activity increases by 1.3 and 1.6fold in rats treated with 10 and 20 mg/kg bezafibrate
thiamine
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thiamine increases the specific activity of the human liver enzyme complex
alpha-Chloroisocaproate
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activation of the enzyme complex in intact cell and tissue preparations
alpha-Chloroisocaproate
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activates through release of branched-chain alpha-keto acid dehydrogenase kinase from the BCKD complex
alpha-Ketoisocaproate
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activates through release of branched-chain alpha-keto acid dehydrogenase kinase from the BCKD complex
alpha-Ketoisocaproate
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promotes dissociation of the BCKDH kinase from the BCKDH complex, consistent with activation of BCKDH complex
L-Val
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activates, when ommited from the assay mixture the rate of NAD+ reduction decreases to 41% of the rate of the complete system
Lipoamide
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the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
Lipoamide
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the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
Lipoamide
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the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
thiamine diphosphate
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thiamine diphosphate
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the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
thiamine diphosphate
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Km: 0.00035 mM in the reaction with 4-methyl-2-oxopentanoate
thiamine diphosphate
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the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
thiamine diphosphate
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thiamine diphosphate
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the multienzyme complex catalyzes a thiamine diphosphate- and Mg2+-dependent oxidative decarboxylation of branched-chain keto acids with the formation of branched-chain acyl-CoA and reduction of NAD+ to NADH: R1R2CHCOCOOH + NAD+ + CoASH = R1R2CHCO-S-CoA + NADH + CO2, component E1 (EC 1.2.4.4, branched-chain keto acid dehydrogenase), component E2 (no EC number, branched-chain acyltransferase), component E3 (EC 1.8.1.4, dihydrolipoamide dehydrogenase), partial reactions catalyzed by the multienzyme complex: 1. R1R2CHCOCOOH + E1-(thiamine diphosphate) = E1-(thiamine diphosphate)-COHCHR1R2 + CO2, 2. E1-(thiamine diphosphate)-COHCHR1R2 + lipoyl-E2 = R1R2CHCO-SCoA + dihydrolipoyl-E2, 4. dihydrolipoyl-E2 + NAD+ = lipoyl-E2 + NADH
thiamine diphosphate
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Km: 0.0012 mM in the reaction with 3-methyl-2-oxobutanoate
tumor necrosis factor-alpha
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activates the multienzyme complex about 4fold at 0.05 mg/kg body weight 4 h prior to slaughtering, reduces the amount of phosphorylated E1b component
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tumor necrosis factor-alpha
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0.025 or 0.5 mg per kg body weight elevate the activity state of BCKDH complex from 22% to 69% and 86%, respectively, decrease in the bound form of BCKDH kinase is involved in the mechanism responsible for tumor necrosis factor-alpha induced activation of the BCKDH complex
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tumor necrosis factor-alpha
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promotes dissociation of the BCKDH kinase from the BCKDH complex in rat liver, resulting in activation of the BCKDH complex
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additional information
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clofibric acid and thiamine diphosphate have no effect on the protein-protein interaction between branched-chain alpha-keto acid dehydrogenase kinase and BCKD complex
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