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Literature summary extracted from

  • Paxton, R.
    Branched-chain alpha-keto acid dehydrogenase and its kinase from rabbit liver and heart (1988), Methods Enzymol., 166, 313-320.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
1.2.4.4 thiamine diphosphate
-
Oryctolagus cuniculus

Inhibitors

EC Number Inhibitors Comment Organism Structure
2.7.11.4 2-Chloroisohexanoate
-
Bos taurus
2.7.11.4 2-Chloroisohexanoate
-
Oryctolagus cuniculus
2.7.11.4 2-Chloroisohexanoate
-
Rattus norvegicus
2.7.11.4 2-oxo-3-methylpentanoate
-
Oryctolagus cuniculus
2.7.11.4 2-oxo-3-methylpentanoate
-
Rattus norvegicus
2.7.11.4 2-oxobutanoate
-
Oryctolagus cuniculus
2.7.11.4 2-oxohexanedioate
-
Bos taurus
2.7.11.4 2-oxohexanedioate
-
Oryctolagus cuniculus
2.7.11.4 2-oxohexanedioate
-
Rattus norvegicus
2.7.11.4 2-Oxohexanoate
-
Oryctolagus cuniculus
2.7.11.4 2-oxoisocaproate
-
Bos taurus
2.7.11.4 2-oxoisocaproate
-
Oryctolagus cuniculus
2.7.11.4 2-oxoisocaproate
-
Rattus norvegicus
2.7.11.4 2-oxoisopentanoate
-
Oryctolagus cuniculus
2.7.11.4 2-oxoisopentanoate
-
Rattus norvegicus
2.7.11.4 2-oxopentanoate
-
Bos taurus
2.7.11.4 2-oxopentanoate
-
Oryctolagus cuniculus
2.7.11.4 4-hydroxyphenylpyruvate
-
Oryctolagus cuniculus
2.7.11.4 acetoacetyl-CoA
-
Bos taurus
2.7.11.4 acetoacetyl-CoA
-
Oryctolagus cuniculus
2.7.11.4 acetoacetyl-CoA
-
Rattus norvegicus
2.7.11.4 ADP
-
Bos taurus
2.7.11.4 ADP competitive Oryctolagus cuniculus
2.7.11.4 ADP
-
Rattus norvegicus
2.7.11.4 Ca2+ weak Oryctolagus cuniculus
2.7.11.4 Clofibric acid
-
Oryctolagus cuniculus
2.7.11.4 Dichloroacetate
-
Oryctolagus cuniculus
2.7.11.4 Dichloroacetate
-
Rattus norvegicus
2.7.11.4 heparin
-
Oryctolagus cuniculus
2.7.11.4 isobutyryl-CoA
-
Oryctolagus cuniculus
2.7.11.4 isovaleryl-CoA
-
Oryctolagus cuniculus
2.7.11.4 malonyl-CoA
-
Oryctolagus cuniculus
2.7.11.4 methylmalonyl-CoA
-
Bos taurus
2.7.11.4 methylmalonyl-CoA
-
Oryctolagus cuniculus
2.7.11.4 methylmalonyl-CoA
-
Rattus norvegicus
2.7.11.4 Mg2+ at concentrations above 1.5 mM, activation below Oryctolagus cuniculus
2.7.11.4 n-Octanoate
-
Bos taurus
2.7.11.4 n-Octanoate
-
Oryctolagus cuniculus
2.7.11.4 n-Octanoate
-
Rattus norvegicus
2.7.11.4 NADP+
-
Oryctolagus cuniculus
2.7.11.4 phenylacetate
-
Oryctolagus cuniculus
2.7.11.4 Phenyllactate
-
Oryctolagus cuniculus
2.7.11.4 pyruvate
-
Oryctolagus cuniculus

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
2.7.11.4 0.025
-
ATP pH 7.35, 20°C Oryctolagus cuniculus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
2.7.11.4 mitochondrion part of intramitochondrial branched-chain 2-oxoacid dehydrogenase complex Rattus norvegicus 5739
-
2.7.11.4 mitochondrion part of intramitochondrial branched-chain 2-oxoacid dehydrogenase complex Bos taurus 5739
-
2.7.11.4 mitochondrion part of intramitochondrial branched-chain 2-oxoacid dehydrogenase complex Oryctolagus cuniculus 5739
-

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
2.7.11.4 EGTA activation, presumably by chelation of Ca2+ Oryctolagus cuniculus
2.7.11.4 Mg2+
-
Rattus norvegicus
2.7.11.4 Mg2+
-
Bos taurus
2.7.11.4 Mg2+ maximum activity at 1.5 mM, inhibits above 1.5 mM Oryctolagus cuniculus

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
2.7.11.4 2000000
-
above 2000000, gel filtration Oryctolagus cuniculus

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] Rattus norvegicus phosphorylation inactivates EC 1.2.4.4 ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] Bos taurus phosphorylation inactivates EC 1.2.4.4 ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] Oryctolagus cuniculus phosphorylation inactivates EC 1.2.4.4 ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?

Organism

EC Number Organism UniProt Comment Textmining
1.2.4.4 Oryctolagus cuniculus
-
-
-
2.7.11.4 Bos taurus
-
-
-
2.7.11.4 Oryctolagus cuniculus
-
-
-
2.7.11.4 Rattus norvegicus
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
2.7.11.4
-
Rattus norvegicus
2.7.11.4 alpha-ketoacid dehydrogenase complex Oryctolagus cuniculus

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.2.4.4 liver
-
Oryctolagus cuniculus
-
2.7.11.4 brain
-
Oryctolagus cuniculus
-
2.7.11.4 heart
-
Rattus norvegicus
-
2.7.11.4 kidney
-
Rattus norvegicus
-
2.7.11.4 kidney
-
Bos taurus
-
2.7.11.4 kidney cortex Oryctolagus cuniculus
-
2.7.11.4 liver
-
Rattus norvegicus
-
2.7.11.4 liver
-
Bos taurus
-
2.7.11.4 liver
-
Oryctolagus cuniculus
-
2.7.11.4 skeletal muscle
-
Rattus norvegicus
-
2.7.11.4 skeletal muscle
-
Oryctolagus cuniculus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.2.4.4 2-oxo-4-methylthiobutanoate + NAD+ + CoA
-
Oryctolagus cuniculus 3-methylthiopropionyl-CoA + NADH + CO2
-
?
1.2.4.4 2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine
-
Oryctolagus cuniculus [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(propanoyl)dihydrolipoyllysine + CO2
-
?
1.2.4.4 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine
-
Oryctolagus cuniculus [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2
-
?
1.2.4.4 3-methyl-2-oxopentanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine
-
Oryctolagus cuniculus [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylbutanoyl)dihydrolipoyllysine + CO2
-
?
1.2.4.4 4-methyl-2-oxopentanoate + NAD+ + CoA
-
Oryctolagus cuniculus 3-methylbutanoyl-CoA + CO2 + NADH
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] 2 Ser-residues in E1-alpha-subunit Rattus norvegicus ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] 2 Ser-residues in E1-alpha-subunit Bos taurus ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] 2 Ser-residues in E1-alpha-subunit Oryctolagus cuniculus ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] incorporates 0.75 mol phosphate per mol phosphorylation site and 1.5 mol/mol alpha-subunit Oryctolagus cuniculus ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphorylation inactivates EC 1.2.4.4 Rattus norvegicus ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphorylation inactivates EC 1.2.4.4 Bos taurus ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?
2.7.11.4 ATP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphorylation inactivates EC 1.2.4.4 Oryctolagus cuniculus ADP + [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] phosphate
-
?

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
2.7.11.4 additional information
-
in decreasing order of activity: HEPES, potassium phosphate, imidazole, 3-(N-morpholino)ethane buffer Oryctolagus cuniculus
2.7.11.4 7.5
-
-
Oryctolagus cuniculus

Cofactor

EC Number Cofactor Comment Organism Structure
1.2.4.4 CoA
-
Oryctolagus cuniculus
1.2.4.4 NAD+
-
Oryctolagus cuniculus

Ki Value [mM]

EC Number Ki Value [mM] Ki Value maximum [mM] Inhibitor Comment Organism Structure
2.7.11.4 0.13
-
ADP pH 7.35, 20°C Oryctolagus cuniculus