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(indol-3-yl)pyruvate
(indol-3-yl)acetaldehyde + CO2
2-oxo-3-methylpentanoate
2-methylbutanal + CO2
Substrates: -
Products: -
?
2-oxo-3-methylvalerate
2-methylbutanal + CO2
-
Substrates: the enzymatic activity toward 2-oxo-3-methylvalerate is 1.5times higher than the activity toward 2-oxo-isovalerate
Products: -
?
2-oxo-3-phenylpropanoic acid
phenylacetaldehyde + CO2
-
Substrates: at 30 mM 8.6% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
2-oxobutanoate
propanal + CO2
Substrates: -
Products: -
?
2-oxobutanoic acid
propanal + CO2
2-oxobutyrate
propionaldehyde + CO2
Substrates: -
Products: -
?
2-oxoglutarate
CO2 + succinate semialdehyde
-
Substrates: 5% of the activity with L-3-methyl-2-oxopentanoate
Products: -
?
2-oxohexanoate
n-pentanal + CO2
Substrates: -
Products: -
?
2-oxohexanoic acid
pentanal + CO2
2-oxoisocaproate
3-methylbutanal + CO2
2-oxoisohexanoate
? + CO2
Substrates: -
Products: -
?
2-oxoisohexanoate
CO2 + isopentanal
2-oxoisopentanoate
2-methylpropanal + CO2
Substrates: -
Products: -
?
2-oxoisopentanoate
CO2 + isobutanal
-
Substrates: 63% of the activity with L-3-methyl-
Products: -
?
2-oxoisovalerate
2-methylpropanal + CO2
2-oxoisovalerate
isobutanal + CO2
2-oxomethylvalerate
pentanal + CO2
2-oxopentanoate
butanal + CO2
Substrates: -
Products: -
?
2-oxopentanoate
n-butanal + CO2
Substrates: -
Products: -
?
2-oxopentanoic acid
butanal + CO2
2-oxopropanoic acid
acetaldehyde + CO2
-
Substrates: at 30 mM 1.2% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
3,5-dimethoxybenzaldehyde + 2-oxopropanoic acid
(1R)-1-(3,5-dimethoxyphenyl)-1-hydroxypropan-2-one + CO2
-
Substrates: -
Products: -
?
3-(4-hydroxyphenyl)-2-oxopropanoic acid
(4-hydroxyphenyl)-acetaldehyde + CO2
-
Substrates: at 30 mM 6% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
3-formylbenzonitrile + 2-oxobutanoic acid
3-[(1R)-1-hydroxy-2-oxobutyl]benzonitrile
-
Substrates: -
Products: -
?
3-methyl-2-oxobutanoate
2-methylpropanal + CO2
3-methyl-2-oxobutanoic acid
2-methylpropanal + CO2
3-methyl-2-oxobutyrate
3-methylpropanal + CO2
3-methyl-2-oxopentanoate
2-methylbutanal + CO2
3-methyl-2-oxopentanoate
CO2 + 2-methylbutanal
3-methyl-2-oxopentanoic acid
2-methylbutanal + CO2
4-(4-hydroxyphenyl)-2-oxobutanoic acid
3-(4-hydroxyphenyl)-propanal + CO2
-
Substrates: at 30 mM 1.6% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
4-(methylsulfanyl)-2-oxobutanoic acid
3-(methylsulfanyl)-propanal + CO2
-
Substrates: at 30 mM 18% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
4-hydroxyphenylpyruvate
4-hydroxyphenylacetaldehyde + CO2
4-methyl-2-oxohexanoic acid
3-methylpentanal + CO2
-
Substrates: at 30 mM 19% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
4-methyl-2-oxopentanoate
4-methylbutanal + CO2
4-methyl-2-oxopentanoic acid
3-methylbutanal + CO2
4-methylthio-2-oxobutanoate
3-methylthiopropanal + CO2
4-methylthio-2-oxobutanoic acid
3-methylthiopropanal + CO2
Substrates: 10% of the rate with 3-methyl-2-oxobutanoic acid
Products: -
?
5-(4-hydroxyphenyl)-2-oxopentanoic acid
4-(4-hydroxyphenyl)-butanal + CO2
-
Substrates: at 30 mM 1.1% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
alpha-isocaproic acid
3-methylbutanal + CO2
Substrates: -
Products: -
?
benzaldehyde + CO2
(R)-benzoin
-
Substrates: self-carboligation
Products: -
?
benzoylformate
? + CO2
Substrates: -
Products: -
?
indole-3-pyruvate
?
-
Substrates: at 1 mM 0.85% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
isovaleraldehyde
?
-
Substrates: self-carboligation
Products: -
?
L-3-methyl-2-oxopentanoate
CO2 + 2-methylbutanal
oxo(phenyl)acetic acid
benzaldehyde + CO2
-
Substrates: at 30 mM 8.4% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
pyruvate
acetaldehyde + CO2
additional information
?
-
(indol-3-yl)pyruvate
(indol-3-yl)acetaldehyde + CO2
Substrates: -
Products: -
?
(indol-3-yl)pyruvate
(indol-3-yl)acetaldehyde + CO2
Substrates: -
Products: -
?
2-oxobutanoic acid
propanal + CO2
Substrates: 10% of the rate with 3-methyl-2-oxobutanoic acid
Products: -
?
2-oxobutanoic acid
propanal + CO2
-
Substrates: at 30 mM 9.3% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
2-oxohexanoic acid
pentanal + CO2
Substrates: 30% of the rate with 3-methyl-2-oxobutanoic acid
Products: -
?
2-oxohexanoic acid
pentanal + CO2
-
Substrates: at 30 mM 13% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
2-oxoisocaproate
3-methylbutanal + CO2
Substrates: -
Products: -
?
2-oxoisocaproate
3-methylbutanal + CO2
Substrates: -
Products: -
?
2-oxoisocaproate
3-methylbutanal + CO2
Substrates: 22.7% of the activity with 2-oxoisovalerate
Products: -
?
2-oxoisohexanoate
CO2 + isopentanal
-
Substrates: 38% of the activity with L-3-methyl-2-oxopentanoate
Products: -
?
2-oxoisohexanoate
CO2 + isopentanal
-
Substrates: 38% of the activity with L-3-methyl-2-oxopentanoate
Products: -
?
2-oxoisovalerate
2-methylpropanal + CO2
-
Substrates: -
Products: -
?
2-oxoisovalerate
2-methylpropanal + CO2
-
Substrates: -
Products: -
?
2-oxoisovalerate
2-methylpropanal + CO2
Substrates: -
Products: -
?
2-oxoisovalerate
2-methylpropanal + CO2
Substrates: -
Products: -
?
2-oxoisovalerate
2-methylpropanal + CO2
Substrates: -
Products: -
?
2-oxoisovalerate
isobutanal + CO2
Substrates: -
Products: -
?
2-oxoisovalerate
isobutanal + CO2
Substrates: -
Products: -
?
2-oxomethylvalerate
pentanal + CO2
Substrates: -
Products: -
?
2-oxomethylvalerate
pentanal + CO2
Substrates: 16.7% of the activity with 2-oxoisovalerate
Products: -
?
2-oxopentanoic acid
butanal + CO2
Substrates: 25% of the rate with 3-methyl-2-oxobutanoic acid
Products: -
?
2-oxopentanoic acid
butanal + CO2
-
Substrates: at 30 mM 15% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
3-methyl-2-oxobutanoate
2-methylpropanal + CO2
Substrates: -
Products: -
?
3-methyl-2-oxobutanoate
2-methylpropanal + CO2
Substrates: -
Products: -
?
3-methyl-2-oxobutanoic acid
2-methylpropanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxobutanoic acid
2-methylpropanal + CO2
Substrates: -
Products: -
?
3-methyl-2-oxobutanoic acid
2-methylpropanal + CO2
-
Substrates: 100% activity
Products: -
?
3-methyl-2-oxobutyrate
3-methylpropanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxobutyrate
3-methylpropanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxobutyrate
3-methylpropanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxobutyrate
3-methylpropanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxobutyrate
3-methylpropanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxopentanoate
2-methylbutanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxopentanoate
2-methylbutanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxopentanoate
2-methylbutanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxopentanoate
2-methylbutanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxopentanoate
2-methylbutanal + CO2
-
Substrates: -
Products: -
?
3-methyl-2-oxopentanoate
2-methylbutanal + CO2
Substrates: -
Products: -
?
3-methyl-2-oxopentanoate
CO2 + 2-methylbutanal
-
Substrates: -
Products: -
?
3-methyl-2-oxopentanoate
CO2 + 2-methylbutanal
-
Substrates: -
Products: -
?
3-methyl-2-oxopentanoic acid
2-methylbutanal + CO2
Substrates: 30% of the rate with 3-methyl-2-oxobutanoic acid
Products: -
?
3-methyl-2-oxopentanoic acid
2-methylbutanal + CO2
-
Substrates: at 30 mM 38.1% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
4-hydroxyphenylpyruvate
4-hydroxyphenylacetaldehyde + CO2
Substrates: -
Products: -
?
4-hydroxyphenylpyruvate
4-hydroxyphenylacetaldehyde + CO2
Substrates: -
Products: -
?
4-methyl-2-oxopentanoate
4-methylbutanal + CO2
-
Substrates: -
Products: -
?
4-methyl-2-oxopentanoate
4-methylbutanal + CO2
-
Substrates: -
Products: -
?
4-methyl-2-oxopentanoate
4-methylbutanal + CO2
-
Substrates: -
Products: -
?
4-methyl-2-oxopentanoate
4-methylbutanal + CO2
-
Substrates: -
Products: -
?
4-methyl-2-oxopentanoate
4-methylbutanal + CO2
-
Substrates: -
Products: -
?
4-methyl-2-oxopentanoate
4-methylbutanal + CO2
Substrates: -
Products: -
?
4-methyl-2-oxopentanoic acid
3-methylbutanal + CO2
Substrates: 35% of the rate with 3-methyl-2-oxobutanoic acid
Products: -
?
4-methyl-2-oxopentanoic acid
3-methylbutanal + CO2
-
Substrates: at 30 mM 26.3% activity relative to 3-methyl-2-oxobutanoic acid
Products: -
?
4-methylthio-2-oxobutanoate
3-methylthiopropanal + CO2
Substrates: -
Products: -
?
4-methylthio-2-oxobutanoate
3-methylthiopropanal + CO2
Substrates: -
Products: -
?
L-3-methyl-2-oxopentanoate
CO2 + 2-methylbutanal
-
Substrates: stereospecificity towards the L-isomer
Products: -
?
L-3-methyl-2-oxopentanoate
CO2 + 2-methylbutanal
-
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
-
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
Substrates: -
Products: 8.8% of the activity with 2-oxoisovalerate
?
Phenylpyruvate
Phenylacetaldehyde + CO2
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
-
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
-
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
-
Substrates: -
Products: -
?
Phenylpyruvate
Phenylacetaldehyde + CO2
-
Substrates: -
Products: -
?
pyruvate
acetaldehyde + CO2
Substrates: -
Products: -
?
pyruvate
acetaldehyde + CO2
Substrates: -
Products: -
?
pyruvate
acetaldehyde + CO2
Substrates: -
Products: -
?
pyruvate
acetaldehyde + CO2
Substrates: -
Products: -
?
pyruvate
acetaldehyde + CO2
Substrates: -
Products: -
?
pyruvate
ethanal + CO2
-
Substrates: 25% of the activity with L-3-methyl-2-oxopentanoate
Products: -
?
pyruvate
ethanal + CO2
-
Substrates: -
Products: -
?
pyruvate
ethanal + CO2
-
Substrates: -
Products: -
?
pyruvate
ethanal + CO2
-
Substrates: -
Products: -
?
pyruvate
ethanal + CO2
-
Substrates: -
Products: -
?
pyruvate
ethanal + CO2
-
Substrates: -
Products: -
?
additional information
?
-
-
Substrates: essential for the synthesis of branched-chain fatty acids
Products: -
?
additional information
?
-
-
Substrates: plants with enzymic activity show enhanced cold tolerance, role as a protective mechanism for growth of plants under sub optimal temperatures
Products: -
?
additional information
?
-
Substrates: acetolactate synthase AlsS, EC 2.2.1.6, is able to catalyze the decarboxylation of 2-oxoisovalerate both in vivo and in vitro
Products: -
?
additional information
?
-
Substrates: acetolactate synthase AlsS, EC 2.2.1.6, is able to catalyze the decarboxylation of 2-oxoisovalerate both in vivo and in vitro
Products: -
?
additional information
?
-
-
Substrates: 3-fluoro-2-oxopropanoic acid, isobutyraldehyde, 3,5-dichlorobenzaldehyde, 3,5-dimethoxybenzaldehyde and 2-oxooctanoic acid are no substrates
Products: -
?
additional information
?
-
-
Substrates: the enzyme is also able to catalyze carboligation reactions with an exceptionally broad substrate range, a feature that makes KdcA a potentially valuable biocatalyst for C-C bond formation, in particular for the enzymatic synthesis of diversely substituted 2-hydroxyketones with high enantioselectivity
Products: -
?
additional information
?
-
-
Substrates: enzyme additionallly catalyzes the asymmetric synthesis of chiral 2-hydroxy ketones with high stereochemical purity
Products: -
?
additional information
?
-
Substrates: very poor substrates: indole-3-pyruvate and pyruvate
Products: -
?
additional information
?
-
Substrates: enzyme is found to convert a broad spectrum of branched-chain and aromatic alpha-keto acids. Determined the catalytic constants for the conversion of 11 aliphatic, aromatic, and branched-chain alpha-keto acids
Products: -
?
additional information
?
-
-
Substrates: enzyme is found to convert a broad spectrum of branched-chain and aromatic alpha-keto acids. Determined the catalytic constants for the conversion of 11 aliphatic, aromatic, and branched-chain alpha-keto acids
Products: -
?
additional information
?
-
Substrates: enzyme is found to convert a broad spectrum of branched-chain and aromatic alpha-keto acids. Determined the catalytic constants for the conversion of 11 aliphatic, aromatic, and branched-chain alpha-keto acids
Products: -
?
additional information
?
-
-
Substrates: enzyme is found to convert a broad spectrum of branched-chain and aromatic alpha-keto acids. Determined the catalytic constants for the conversion of 11 aliphatic, aromatic, and branched-chain alpha-keto acids
Products: -
?
additional information
?
-
-
Substrates: mitochondrial branched-chain aminotransferase binds to the E1 decarboxylase of BCKDC, forming a metabolon that allows channeling of branched-chain alpha-keto acids from mitochondrial branched-chain aminotransferase to E1
Products: -
?
additional information
?
-
Substrates: no activity with 2-oxoglutarate
Products: -
?
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Acidosis
Acidosis and glucocorticoids induce branched-chain amino acid catabolism.
Acidosis
Chronic metabolic acidosis accelerates whole body proteolysis and oxidation in awake rats.
Acidosis
Glucocorticoids and acidification independently increase transcription of branched-chain ketoacid dehydrogenase subunit genes.
Acidosis
Mechanisms contributing to muscle-wasting in acute uremia: activation of amino acid catabolism.
Acidosis
Mechanisms for protein catabolism in uremia: metabolic acidosis and activation of proteolytic pathways.
Acidosis
Metabolic acidosis accelerates whole body protein degradation and leucine oxidation by a glucocorticoid-dependent mechanism.
Acidosis
Tissue-specific responses of branched-chain alpha-ketoacid dehydrogenase activity in metabolic acidosis.
Brain Diseases
Living donor liver transplantation in maple syrup urine disease - Case series and world's youngest domino liver donor and recipient.
branched-chain-2-oxoacid decarboxylase deficiency
Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites.
Carcinoma, Hepatocellular
Regulation of expression of branched-chain alpha-keto acid dehydrogenase subunits in permanent cell lines.
Maple Syrup Urine Disease
A distinct variant of intermediate maple syrup urine disease.
Maple Syrup Urine Disease
Altered kinetic properties of the branched-chain alpha-keto acid dehydrogenase complex due to mutation of the beta-subunit of the branched-chain alpha-keto acid decarboxylase (E1) component in lymphoblastoid cells derived from patients with maple syrup urine disease.
Maple Syrup Urine Disease
Maple syrup urine disease: branched-chain keto acid decarboxylation in fibroblasts as measured with amino acids and keto acids.
Neoplasms
Administration of endotoxin, tumor necrosis factor, or interleukin 1 to rats activates skeletal muscle branched-chain alpha-keto acid dehydrogenase.
Sepsis
Administration of endotoxin, tumor necrosis factor, or interleukin 1 to rats activates skeletal muscle branched-chain alpha-keto acid dehydrogenase.
Starvation
Effect of starvation on branched-chain alpha-keto acid dehydrogenase activity in rat heart and skeletal muscle.
Tuberculosis
Amino acids allosterically regulate the thiamine diphosphate-dependent alpha-keto acid decarboxylase from Mycobacterium tuberculosis.
Uremia
Glucocorticoids and acidification independently increase transcription of branched-chain ketoacid dehydrogenase subunit genes.
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0.09
(indol-3-yl)pyruvate
-
20.25
2-oxo-3-methylpentanoate
-
0.127
2-oxo-3-phenylpropanoate
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
5.58
2-oxobutanoate
Hill coefficient 1.1, pH 7.0, 30°C
0.6
2-oxohexanoic acid
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
0.57
2-oxoisocaproate
Hill coefficient 1.0, pH 7.0, 30°C
2
2-Oxoisohexanoate
-
25°C, pH 7.2
0.05 - 342
2-oxoisovalerate
3.49
2-oxomethylvalerate
Hill coefficient 0.8, pH 7.0, 30°C
1.21 - 1.44
2-oxopentanoate
1.3
2-oxopentanoic acid
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
29.77
2-Oxopropanoic acid
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
0.63
3-(4-hydroxyphenyl)-2-oxopropanoic acid
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
0.027 - 14.6
3-methyl-2-oxobutanoate
5.02
3-methyl-2-oxobutanoic acid
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
0.1 - 0.87
3-methyl-2-oxopentanoate
1.3
4-(methylsulfanyl)-2-oxobutanoic acid
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
0.042
4-methyl-2-oxopentanoate
pH 7.0, 55°C
0.264
4-methyl-2-oxopentanoic acid
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
1.43
4-methylthio-2-oxobutanoate
Hill coefficient 1.1, pH 7.0, 30°C
0.234
Indole-3-pyruvate
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
0.001
L-3-Methyl-2-oxopentanoate
-
and 2-oxoisopentanoate, 2-oxoisohexanoate, value below, 30°C, pH 7.5
7.5
oxo(phenyl)acetic acid
-
in 50 mM potassium phosphate buffer, pH 6.8 in the presence of 2.5 mM MgSO4 and 0.1 mM thiamine diphosphate
0.2 - 1.17
phenylpyruvate
0.00063 - 0.04
thiamine diphosphate
0.05
2-oxoisovalerate
-
mutant D295A, pH 7.5, 30°C, E1b-catalyzed decarboxylation reaction
0.05
2-oxoisovalerate
-
mutant D295A, pH 7.5, 30°C, overall reaction
0.05
2-oxoisovalerate
-
wild-type, pH 7.5, 30°C, E1b-catalyzed decarboxylation reaction
0.05
2-oxoisovalerate
-
wild-type, pH 7.5, 30°C, overall reaction
0.14
2-oxoisovalerate
-
mutant Y300A, pH 7.5, 30°C, overall reaction
0.225
2-oxoisovalerate
-
mutant R301A, pH 7.5, 30°C, E1b-catalyzed decarboxylation reaction
0.252
2-oxoisovalerate
-
mutant Y300F, pH 7.5, 30°C, E1b-catalyzed decarboxylation reaction
0.255
2-oxoisovalerate
-
mutant R301A, pH 7.5, 30°C, overall reaction
0.337
2-oxoisovalerate
-
mutant Y300A, pH 7.5, 30°C, E1b-catalyzed decarboxylation reaction
0.39
2-oxoisovalerate
-
mutant Y300F, pH 7.5, 30°C, overall reaction
1.9
2-oxoisovalerate
pH 6.5, 37°C
8.31
2-oxoisovalerate
Hill coefficient 0.9, pH 7.0, 30°C
154
2-oxoisovalerate
mutant Q487S, pH 7.0, 37°C
175
2-oxoisovalerate
mutant Q487G, pH 7.0, 37°C
186
2-oxoisovalerate
mutant Q487A, pH 7.0, 37°C
300
2-oxoisovalerate
wild-type, pH 7.0, 37°C
323
2-oxoisovalerate
mutant Q487I, pH 7.0, 37°C
342
2-oxoisovalerate
mutant Q487L, pH 7.0, 37°C