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O55028

- BCKD_MOUSE

UniProt

O55028 - BCKD_MOUSE

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Protein

[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial

Gene
Bckdk
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

Functioni

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 activityi

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

GO - Molecular functioni

  1. [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity Source: UniProtKB-EC
  2. ATP binding Source: HGNC
  3. protein kinase activity Source: MGI
  4. protein serine/threonine kinase activity Source: HGNC

GO - Biological processi

  1. branched-chain amino acid catabolic process Source: HGNC
  2. phosphorylation Source: HGNC
  3. protein phosphorylation Source: GOC
Complete GO annotation...

Keywords - Molecular functioni

Kinase, Serine/threonine-protein kinase, Transferase

Keywords - Ligandi

ATP-binding, Nucleotide-binding

Names & Taxonomyi

Protein namesi
Recommended 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 namesi
Name:Bckdk
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 7

Organism-specific databases

MGIiMGI:1276121. Bckdk.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial alpha-ketoglutarate dehydrogenase complex Source: HGNC
  2. mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Disruption phenotypei

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.2 Publications

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3030Mitochondrion By similarityAdd
BLAST
Chaini31 – 412382[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrialPRO_0000023453Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei52 – 521Phosphoserine; by autocatalysis By similarity
Modified residuei192 – 1921N6-acetyllysine1 Publication
Modified residuei233 – 2331N6-acetyllysine By similarity

Post-translational modificationi

Autophosphorylated By similarity.

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiO55028.
PaxDbiO55028.
PRIDEiO55028.

PTM databases

PhosphoSiteiO55028.

Expressioni

Tissue specificityi

Ubiquitous.

Inductioni

Expression in female liver is influenced by circadian rhythm.

Gene expression databases

ArrayExpressiO55028.
BgeeiO55028.
CleanExiMM_BCKDK.
GenevestigatoriO55028.

Interactioni

Subunit structurei

Monomer By similarity.

Structurei

3D structure databases

ProteinModelPortaliO55028.
SMRiO55028. Positions 68-408.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini159 – 404246Histidine kinaseAdd
BLAST

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0642.
HOGENOMiHOG000164315.
HOVERGENiHBG004829.
InParanoidiO55028.
KOiK00905.
OMAiTIANNDV.
OrthoDBiEOG7TQV0S.
PhylomeDBiO55028.
TreeFamiTF331303.

Family and domain databases

Gene3Di1.20.140.20. 1 hit.
3.30.565.10. 1 hit.
InterProiIPR018955. BCDHK/PDK_N.
IPR003594. HATPase_ATP-bd.
IPR004358. Sig_transdc_His_kin-like_C.
IPR005467. Sig_transdc_His_kinase_core.
[Graphical view]
PfamiPF10436. BCDHK_Adom3. 1 hit.
PF02518. HATPase_c. 1 hit.
[Graphical view]
PRINTSiPR00344. BCTRLSENSOR.
SMARTiSM00387. HATPase_c. 1 hit.
[Graphical view]
SUPFAMiSSF55874. SSF55874. 1 hit.
SSF69012. SSF69012. 1 hit.
PROSITEiPS50109. HIS_KIN. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O55028-1 [UniParc]FASTAAdd to Basket

« Hide

MILTSVLGSG PRSWSSLWPL LGSSLSLRAR STSATDTHHV ELARERSKTV    50
TSFYNQSAID VAAEKPSVRL TPTMMLYSGR SQDGSHLLKS GRYLQQELPV 100
RIAHRIKGFR SLPFIIGCNP TILHVHELYI RAFQKLTDFP PIKDQADEAQ 150
YCQLVRQLLD DHKDVVTLLA EGLRESRKHI QDEKLVRYFL DKTLTSRLGI 200
RMLATHHLAL HEDKPDFVGI ICTRLSPKKI IEKWVDFARR LCEHKYGNAP 250
RVRINGHVAA RFPFIPMPLD YILPELLKNA MRATMESHLD TPYNVPDVVI 300
TIANNDIDLI IRISDRGGGI AHKDLDRVMD YHFTTAEAST QDPRINPLFG 350
HLDMHSGGQS GPMHGFGFGL PTSRAYAEYL GGSLQLQSLQ GIGTDVYLRL 400
RHIDGREESF RI 412
Length:412
Mass (Da):46,588
Last modified:June 1, 1998 - v1
Checksum:i80B3B173AAC8AAD3
GO

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AF043070 mRNA. Translation: AAB97689.1.
BC046595 mRNA. Translation: AAH46595.1.
CCDSiCCDS21884.1.
RefSeqiNP_033869.1. NM_009739.3.
UniGeneiMm.425495.

Genome annotation databases

EnsembliENSMUST00000071056; ENSMUSP00000070345; ENSMUSG00000030802.
GeneIDi12041.
KEGGimmu:12041.
UCSCiuc009jxf.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AF043070 mRNA. Translation: AAB97689.1 .
BC046595 mRNA. Translation: AAH46595.1 .
CCDSi CCDS21884.1.
RefSeqi NP_033869.1. NM_009739.3.
UniGenei Mm.425495.

3D structure databases

ProteinModelPortali O55028.
SMRi O55028. Positions 68-408.
ModBasei Search...
MobiDBi Search...

PTM databases

PhosphoSitei O55028.

Proteomic databases

MaxQBi O55028.
PaxDbi O55028.
PRIDEi O55028.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000071056 ; ENSMUSP00000070345 ; ENSMUSG00000030802 .
GeneIDi 12041.
KEGGi mmu:12041.
UCSCi uc009jxf.1. mouse.

Organism-specific databases

CTDi 10295.
MGIi MGI:1276121. Bckdk.

Phylogenomic databases

eggNOGi COG0642.
HOGENOMi HOG000164315.
HOVERGENi HBG004829.
InParanoidi O55028.
KOi K00905.
OMAi TIANNDV.
OrthoDBi EOG7TQV0S.
PhylomeDBi O55028.
TreeFami TF331303.

Miscellaneous databases

NextBioi 280305.
PROi O55028.
SOURCEi Search...

Gene expression databases

ArrayExpressi O55028.
Bgeei O55028.
CleanExi MM_BCKDK.
Genevestigatori O55028.

Family and domain databases

Gene3Di 1.20.140.20. 1 hit.
3.30.565.10. 1 hit.
InterProi IPR018955. BCDHK/PDK_N.
IPR003594. HATPase_ATP-bd.
IPR004358. Sig_transdc_His_kin-like_C.
IPR005467. Sig_transdc_His_kinase_core.
[Graphical view ]
Pfami PF10436. BCDHK_Adom3. 1 hit.
PF02518. HATPase_c. 1 hit.
[Graphical view ]
PRINTSi PR00344. BCTRLSENSOR.
SMARTi SM00387. HATPase_c. 1 hit.
[Graphical view ]
SUPFAMi SSF55874. SSF55874. 1 hit.
SSF69012. SSF69012. 1 hit.
PROSITEi PS50109. HIS_KIN. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  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. 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.

Entry informationi

Entry nameiBCKD_MOUSE
AccessioniPrimary (citable) accession number: O55028
Entry historyi
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 statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. Human and mouse protein kinases
    Human and mouse protein kinases: classification and index
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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