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O55028 (BCKD_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 125. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial

EC=2.7.11.4
Alternative name(s):
Branched-chain alpha-ketoacid dehydrogenase kinase
Short name=BCKD-kinase
Short name=BCKDHKIN
Gene names
Name:Bckdk
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length412 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex By similarity.

Catalytic activity

ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] = ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate.

Subunit structure

Monomer By similarity.

Subcellular location

Mitochondrion matrix.

Tissue specificity

Ubiquitous.

Induction

Expression in female liver is influenced by circadian rhythm.

Post-translational modification

Autophosphorylated By similarity.

Disruption phenotype

Mutant animals are born at the expected Mendelian ratio, but they exhibit a decreased fertility. They are healthy at birth. At 3 weeks of age, they start showing growth retardation. At 12 weeks, they are 15% smaller than their wild-type littermates. Adults develop neurological abnormalities, such as tremors, epileptic seizures and hindlimb clasping. These neurological deficits can be completely abolished when mice are fed with a diet enriched in branched amino acids. Ref.3 Ref.6

Sequence similarities

Belongs to the PDK/BCKDK protein kinase family.

Contains 1 histidine kinase domain.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 3030Mitochondrion By similarity
Chain31 – 412382[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
PRO_0000023453

Regions

Domain159 – 404246Histidine kinase

Amino acid modifications

Modified residue521Phosphoserine; by autocatalysis By similarity
Modified residue1921N6-acetyllysine Ref.7
Modified residue2331N6-acetyllysine By similarity

Sequences

Sequence LengthMass (Da)Tools
O55028 [UniParc].

Last modified June 1, 1998. Version 1.
Checksum: 80B3B173AAC8AAD3

FASTA41246,588
        10         20         30         40         50         60 
MILTSVLGSG PRSWSSLWPL LGSSLSLRAR STSATDTHHV ELARERSKTV TSFYNQSAID 

        70         80         90        100        110        120 
VAAEKPSVRL TPTMMLYSGR SQDGSHLLKS GRYLQQELPV RIAHRIKGFR SLPFIIGCNP 

       130        140        150        160        170        180 
TILHVHELYI RAFQKLTDFP PIKDQADEAQ YCQLVRQLLD DHKDVVTLLA EGLRESRKHI 

       190        200        210        220        230        240 
QDEKLVRYFL DKTLTSRLGI RMLATHHLAL HEDKPDFVGI ICTRLSPKKI IEKWVDFARR 

       250        260        270        280        290        300 
LCEHKYGNAP RVRINGHVAA RFPFIPMPLD YILPELLKNA MRATMESHLD TPYNVPDVVI 

       310        320        330        340        350        360 
TIANNDIDLI IRISDRGGGI AHKDLDRVMD YHFTTAEAST QDPRINPLFG HLDMHSGGQS 

       370        380        390        400        410 
GPMHGFGFGL PTSRAYAEYL GGSLQLQSLQ GIGTDVYLRL RHIDGREESF RI 

« Hide

References

« Hide 'large scale' references
[1]"Murine branched chain alpha ketoacid dehydrogenase kinase; cDNA cloning, tissue distribution, and temporal expression during embryonic development."
Doering C.B., Coursey C., Spangler W., Danner D.J.
Gene 212:213-219(1998) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
Strain: C57BL/6J.
[2]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Olfactory epithelium.
[3]"Impaired growth and neurological abnormalities in branched-chain alpha-keto acid dehydrogenase kinase-deficient mice."
Joshi M.A., Jeoung N.H., Obayashi M., Hattab E.M., Brocken E.G., Liechty E.A., Kubek M.J., Vattem K.M., Wek R.C., Harris R.A.
Biochem. J. 400:153-162(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: DISRUPTION PHENOTYPE.
[4]"Comprehensive identification of phosphorylation sites in postsynaptic density preparations."
Trinidad J.C., Specht C.G., Thalhammer A., Schoepfer R., Burlingame A.L.
Mol. Cell. Proteomics 5:914-922(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
Tissue: Brain.
[5]"The phagosomal proteome in interferon-gamma-activated macrophages."
Trost M., English L., Lemieux S., Courcelles M., Desjardins M., Thibault P.
Immunity 30:143-154(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[6]"Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy."
Novarino G., El-Fishawy P., Kayserili H., Meguid N.A., Scott E.M., Schroth J., Silhavy J.L., Kara M., Khalil R.O., Ben-Omran T., Ercan-Sencicek A.G., Hashish A.F., Sanders S.J., Gupta A.R., Hashem H.S., Matern D., Gabriel S., Sweetman L. expand/collapse author list , Rahimi Y., Harris R.A., State M.W., Gleeson J.G.
Science 338:394-397(2012) [PubMed] [Europe PMC] [Abstract]
Cited for: DISRUPTION PHENOTYPE.
[7]"Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways."
Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.
Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013) [PubMed] [Europe PMC] [Abstract]
Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-192, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
Tissue: Liver.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AF043070 mRNA. Translation: AAB97689.1.
BC046595 mRNA. Translation: AAH46595.1.
CCDSCCDS21884.1.
RefSeqNP_033869.1. NM_009739.3.
UniGeneMm.425495.

3D structure databases

ProteinModelPortalO55028.
SMRO55028. Positions 68-408.
ModBaseSearch...
MobiDBSearch...

PTM databases

PhosphoSiteO55028.

Proteomic databases

MaxQBO55028.
PaxDbO55028.
PRIDEO55028.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000071056; ENSMUSP00000070345; ENSMUSG00000030802.
GeneID12041.
KEGGmmu:12041.
UCSCuc009jxf.1. mouse.

Organism-specific databases

CTD10295.
MGIMGI:1276121. Bckdk.

Phylogenomic databases

eggNOGCOG0642.
HOGENOMHOG000164315.
HOVERGENHBG004829.
InParanoidO55028.
KOK00905.
OMATIANNDV.
OrthoDBEOG7TQV0S.
PhylomeDBO55028.
TreeFamTF331303.

Gene expression databases

ArrayExpressO55028.
BgeeO55028.
CleanExMM_BCKDK.
GenevestigatorO55028.

Family and domain databases

Gene3D1.20.140.20. 1 hit.
3.30.565.10. 1 hit.
InterProIPR018955. BCDHK/PDK_N.
IPR003594. HATPase_ATP-bd.
IPR004358. Sig_transdc_His_kin-like_C.
IPR005467. Sig_transdc_His_kinase_core.
[Graphical view]
PfamPF10436. BCDHK_Adom3. 1 hit.
PF02518. HATPase_c. 1 hit.
[Graphical view]
PRINTSPR00344. BCTRLSENSOR.
SMARTSM00387. HATPase_c. 1 hit.
[Graphical view]
SUPFAMSSF55874. SSF55874. 1 hit.
SSF69012. SSF69012. 1 hit.
PROSITEPS50109. HIS_KIN. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

NextBio280305.
PROO55028.
SOURCESearch...

Entry information

Entry nameBCKD_MOUSE
AccessionPrimary (citable) accession number: O55028
Entry history
Integrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: June 1, 1998
Last modified: July 9, 2014
This is version 125 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

Human and mouse protein kinases

Human and mouse protein kinases: classification and index

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot