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O55028

- BCKD_MOUSE

UniProt

O55028 - BCKD_MOUSE

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
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    • History
      Entry version 126 (01 Oct 2014)
      Sequence version 1 (01 Jun 1998)
      Previous versions | rss
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    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.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

    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

    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 – 3030MitochondrionBy 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 autocatalysisBy similarity
    Modified residuei192 – 1921N6-acetyllysine1 Publication
    Modified residuei233 – 2331N6-acetyllysineBy 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.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini159 – 404246Histidine kinasePROSITE-ProRule annotationAdd
    BLAST

    Sequence similaritiesi

    Belongs to the PDK/BCKDK protein kinase family.Curated
    Contains 1 histidine kinase domain.PROSITE-ProRule annotation

    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

    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: October 1, 2014
    This is version 126 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