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Information on EC 2.7.11.4 - [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase and Organism(s) Mus musculus and UniProt Accession O55028

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IUBMB Comments
The enzyme has no activating compound but is specific for its substrate. It is a mitochondrial enzyme associated with the branched-chain 2-oxoacid dehydrogenase complex. Phosphorylation inactivates EC 1.2.4.4, 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring).
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Mus musculus
UNIPROT: O55028
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The taxonomic range for the selected organisms is: Mus musculus
The enzyme appears in selected viruses and cellular organisms
Synonyms
bckdk, bckdh kinase, branched-chain alpha-ketoacid dehydrogenase kinase, bckd kinase, branched-chain alpha-keto acid dehydrogenase kinase, bckd-kinase, branched-chain 2-oxo acid dehydrogenase kinase, bcka dehydrogenase kinase, branched chain alpha-ketoacid dehydrogenase kinase, bckdc kinase, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
BCKA dehydrogenase kinase
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BCKD kinase
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BCKDC kinase
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BCKDH kinase
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branched chain alpha-ketoacid dehydrogenase kinase
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branched-chain alpha-ketoacid dehydrogenase kinase
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BCK
-
-
-
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BCKD kinase
-
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BCKDH kinase
-
-
-
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branched-chain 2-oxo acid dehydrogenase kinase
-
-
-
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branched-chain alpha-keto acid dehydrogenase kinase
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-
-
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branched-chain alpha-ketoacid dehydrogenase kinase
branched-chain keto acid dehydrogenase kinase
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-
-
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kinase, branched-chain oxo acid dehydrogenase (phosphorylating)
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
phospho group transfer
-
-
-
-
SYSTEMATIC NAME
IUBMB Comments
ATP:[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphotransferase
The enzyme has no activating compound but is specific for its substrate. It is a mitochondrial enzyme associated with the branched-chain 2-oxoacid dehydrogenase complex. Phosphorylation inactivates EC 1.2.4.4, 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring).
CAS REGISTRY NUMBER
COMMENTARY hide
82391-38-6
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)]
ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate
show the reaction diagram
-
-
-
?
ATP + [branched-chain a-keto acid dehydrogenase (acetyl-transferring)]
ADP + [branched-chain a-keto acid dehydrogenase (acetyl-transferring)] phosphate
show the reaction diagram
-
-
-
?
ATP + branched-chain alpha-keto acid decarboxylase/dehydrogenase
ADP + phosphorylated branched-chain alpha-keto acid decarboxylase/dehydrogenase
show the reaction diagram
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)]
ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate
show the reaction diagram
-
-
-
?
ATP + [branched-chain a-keto acid dehydrogenase (acetyl-transferring)]
ADP + [branched-chain a-keto acid dehydrogenase (acetyl-transferring)] phosphate
show the reaction diagram
-
-
-
?
ATP + branched-chain alpha-keto acid decarboxylase/dehydrogenase
ADP + phosphorylated branched-chain alpha-keto acid decarboxylase/dehydrogenase
show the reaction diagram
-
the catalyzed reversible specific phosphorylation of the BCKD subunit regulates the first committed step in the pathway for leucine, isoleucine, and valine catabolism
-
-
?
COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
(2R)-2-chloro-3-phenylpropanoic acid
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(2S)-2-chloro-3-phenylpropanoic acid
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(R)-alpha-chloroisocaproate
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(S)-alpha-chloroisocaproate
-
2-oxoisocaproate
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3,6-dichlorobenzo[b]thiophene-2-carboxylic acid
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3-chloro-6-fluorobenzo[b]thiophene-2-carboxylic acid
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Clofibric acid
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Hydrocinnamic acid
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methylhydrocinnamic acid
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N,N'-(1,2,5-oxadiazole-3,4-diyl)bis(3,6-dichlorobenzo[b]thiophene-2-carboxamide
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N-(4-acetamido-1,2,5-oxadiazol-3-yl)-3,6-dichlorobenzo[b]thiophene-2-carboxamide
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phenylacetate
-
phenylbutyrate
-
phenylpyruvic acid
-
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
additional information
-
tissue-specific translation of branched-chain alpha-ketoacid dehydrogenase kinase mRNA is dependent upon an upstream open reading frame in the 5'-untranslated region
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IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.0214
(2R)-2-chloro-3-phenylpropanoic acid
Mus musculus
at pH 7.5 and 25°C
0.0063
(2S)-2-chloro-3-phenylpropanoic acid
Mus musculus
at pH 7.5 and 25°C
0.012
(R)-alpha-chloroisocaproate
Mus musculus
at pH 7.5 and 25°C
0.0534
(S)-alpha-chloroisocaproate
Mus musculus
at pH 7.5 and 25°C
0.0749
2-oxoisocaproate
Mus musculus
at pH 7.5 and 25°C
0.00319
3,6-dichlorobenzo[b]thiophene-2-carboxylic acid
Mus musculus
pH and temperature not specified in the publication
0.812
Clofibric acid
Mus musculus
at pH 7.5 and 25°C
0.0431
Hydrocinnamic acid
Mus musculus
at pH 7.5 and 25°C
0.0338
methylhydrocinnamic acid
Mus musculus
at pH 7.5 and 25°C
1.27
phenylacetate
Mus musculus
at pH 7.5 and 25°C
0.0531
phenylbutyrate
Mus musculus
at pH 7.5 and 25°C
0.346
phenylpyruvic acid
Mus musculus
at pH 7.5 and 25°C
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
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liver cell line
Manually annotated by BRENDA team
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undifferentiated myoblast cell line
Manually annotated by BRENDA team
-
-
Manually annotated by BRENDA team
-
fibroblast cell line
Manually annotated by BRENDA team
additional information
-
tissue distribution, tissue-specific translation of branched-chain alpha-ketoacid dehydrogenase kinase mRNA is dependent upon an upstream open reading frame in the 5'-untranslated region, thus mRNA and protein level do not correlate
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
the endurance performance enhanced by 2 weeks of running training is somewhat, but significantly less in BDK-mKO mice than in control mice. Skeletal muscle of BDK-mKO mice has low levels of glycogen. Catabolism of branched-chain amino acids (BCAAs) is greatly enhanced in the muscle of BDK-mKO mice and produces branched-chain acyl-carnitine, which induces perturbation of energy metabolism in the muscle
physiological function
BCKDk contains multiple epitopes that can induce myocarditis. The BCKDk 111-130 is the primary epitope that induces both autoimmune myocarditis and hepatitis in A/J mice. The disease induced by BCKDk peptides can serve as a useful model to study the autoimmune events of inflammatory heart and liver diseases
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
BCKD_MOUSE
412
0
46588
Swiss-Prot
Mitochondrion (Reliability: 2)
CRYSTALLIZATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
in complex with (S)-alpha-chloro-phenylpropionic acid, hanging drop vapor diffusion method, using 12% (w/v) PEG-8000, 0.1 M Tris·HCl, pH 8.5, 1.3-1.5 M NaCl
in complex with 3,6-dichlorobenzo[b]thiophene-2-carboxylic acid
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
DNA sequence determination and analysis, promotor region footprinting, expression analysis, mechanism of the enzyme expression regulation
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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
overexpression of peroxisome proliferator-activated receptor gamma coactivator-1alpha does not increase the expression of the enzyme
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Doering, C.B.; Coursey, C.; Spangler, W.; Danner, D.J.
Murine branched chain alpha-ketoacid dehydrogenase kinase; cDNA cloning, tissue distribution, and temporal expression during embryonic development
Gene
212
213-219
1998
Mus musculus, Mus musculus C57/BL6J
Manually annotated by BRENDA team
Doering, C.B.; Danner, D.J.
Expression of murine branched-chain alpha-keto acid dehydrogenase kinase
Methods Enzymol.
324
491-497
2000
Mus musculus, Mus musculus C57/BL6J
Manually annotated by BRENDA team
Muller, E.A.; Danner, D.J.
Tissue-specific translation of murine branched-chain alpha-ketoacid dehydrogenase kinase mRNA is dependent upon an upstream open reading frame in the 5'-untranslated region
J. Biol. Chem.
279
44645-44655
2004
Mus musculus
Manually annotated by BRENDA team
Tso, S.C.; Gui, W.J.; Wu, C.Y.; Chuang, J.L.; Qi, X.; Skvora, K.J.; Dork, K.; Wallace, A.L.; Morlock, L.K.; Lee, B.H.; Hutson, S.M.; Strom, S.C.; Williams, N.S.; Tambar, U.K.; Wynn, R.M.; Chuang, D.T.
Benzothiophene carboxylate derivatives as novel allosteric inhibitors of branched-chain alpha-ketoacid dehydrogenase kinase
J. Biol. Chem.
289
20583-20593
2014
Homo sapiens (Q14874), Homo sapiens, Mus musculus (O55028), Mus musculus
Manually annotated by BRENDA team
Hatazawa, Y.; Tadaishi, M.; Nagaike, Y.; Morita, A.; Ogawa, Y.; Ezaki, O.; Takai-Igarashi, T.; Kitaura, Y.; Shimomura, Y.; Kamei, Y.; Miura, S.
PGC-1alpha-mediated branched-chain amino acid metabolism in the skeletal muscle
PLoS ONE
9
e91006
2014
Mus musculus (O55028), Mus musculus
Manually annotated by BRENDA team
Tso, S.C.; Qi, X.; Gui, W.J.; Chuang, J.L.; Morlock, L.K.; Wallace, A.L.; Ahmed, K.; Laxman, S.; Campeau, P.M.; Lee, B.H.; Hutson, S.M.; Tu, B.P.; Williams, N.S.; Tambar, U.K.; Wynn, R.M.; Chuang, D.T.
Structure-based design and mechanisms of allosteric inhibitors for mitochondrial branched-chain alpha-ketoacid dehydrogenase kinase
Proc. Natl. Acad. Sci. USA
110
9728-9733
2013
Mus musculus (O55028)
Manually annotated by BRENDA team
Krishnan, B.; Massilamany, C.; Basavalingappa, R.H.; Gangaplara, A.; Kang, G.; Li, Q.; Uzal, F.A.; Strande, J.L.; Delhon, G.A.; Riethoven, J.J.; Steffen, D.; Reddy, J.
Branched chain alpha-ketoacid dehydrogenase kinase 111-130, a T cell epitope that induces both autoimmune myocarditis and hepatitis in A/J mice
Immun. Inflamm. Dis.
5
421-434
2017
Mus musculus (O55028)
Manually annotated by BRENDA team
Xu, M.; Kitaura, Y.; Ishikawa, T.; Kadota, Y.; Terai, C.; Shindo, D.; Morioka, T.; Ota, M.; Morishita, Y.; Ishihara, K.; Shimomura, Y.
Endurance performance and energy metabolism during exercise in mice with a muscle-specific defect in the control of branched-chain amino acid catabolism
PLoS ONE
12
e0180989
2017
Mus musculus (O55028)
Manually annotated by BRENDA team