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Q9CVD2

- ATX3_MOUSE

UniProt

Q9CVD2 - ATX3_MOUSE

Protein

Ataxin-3

Gene

Atxn3

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 106 (01 Oct 2014)
      Sequence version 2 (28 Feb 2003)
      Previous versions | rss
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    Functioni

    Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription.1 Publication

    Catalytic activityi

    Thiol-dependent hydrolysis of ester, thioester, amide, peptide and isopeptide bonds formed by the C-terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal).

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Active sitei14 – 141Nucleophile
    Active sitei119 – 1191Proton acceptorPROSITE-ProRule annotation
    Active sitei134 – 1341PROSITE-ProRule annotation

    GO - Molecular functioni

    1. omega peptidase activity Source: InterPro
    2. protein binding Source: MGI
    3. RNA polymerase II regulatory region DNA binding Source: Ensembl
    4. ubiquitin protein ligase binding Source: UniProtKB
    5. ubiquitin-specific protease activity Source: UniProtKB
    6. ubiquitin thiolesterase activity Source: UniProtKB

    GO - Biological processi

    1. cellular response to heat Source: MGI
    2. cellular response to misfolded protein Source: UniProtKB
    3. exploration behavior Source: MGI
    4. histone H3 deacetylation Source: Ensembl
    5. misfolded or incompletely synthesized protein catabolic process Source: UniProtKB
    6. monoubiquitinated protein deubiquitination Source: UniProtKB
    7. proteasome-mediated ubiquitin-dependent protein catabolic process Source: UniProtKB
    8. regulation of cell-substrate adhesion Source: MGI
    9. regulation of transcription, DNA-templated Source: UniProtKB-KW
    10. transcription, DNA-templated Source: UniProtKB-KW
    11. ubiquitin-dependent protein catabolic process Source: MGI

    Keywords - Molecular functioni

    Hydrolase, Protease, Thiol protease

    Keywords - Biological processi

    Transcription, Transcription regulation, Ubl conjugation pathway

    Protein family/group databases

    MEROPSiC86.001.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Ataxin-3 (EC:3.4.19.12)
    Alternative name(s):
    Machado-Joseph disease protein 1 homolog
    Gene namesi
    Name:Atxn3
    Synonyms:Mjd
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
    ProteomesiUP000000589: Chromosome 12

    Organism-specific databases

    MGIiMGI:1099442. Atxn3.

    Subcellular locationi

    Nucleus By similarity

    GO - Cellular componenti

    1. cytoplasm Source: MGI
    2. mitochondrial matrix Source: MGI
    3. mitochondrial membrane Source: MGI
    4. nuclear inclusion body Source: MGI
    5. nucleus Source: MGI

    Keywords - Cellular componenti

    Nucleus

    Pathology & Biotechi

    Mutagenesis

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Mutagenesisi14 – 141C → A: Abolishes deubiquitinating activity. 1 Publication

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 355355Ataxin-3PRO_0000053832Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Cross-linki1 – 1Peptide (Met-Gly) (interchain with G-Cter in ubiquitin)By similarity
    Cross-linki200 – 200Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)By similarity
    Modified residuei219 – 2191Phosphoserine1 Publication
    Modified residuei268 – 2681Phosphoserine1 Publication
    Modified residuei273 – 2731Phosphoserine1 Publication

    Post-translational modificationi

    Monoubiquitinated by UBE2W, possibly leading to activate the deubiquitinating enzyme activity.

    Keywords - PTMi

    Isopeptide bond, Phosphoprotein, Ubl conjugation

    Proteomic databases

    MaxQBiQ9CVD2.
    PaxDbiQ9CVD2.
    PRIDEiQ9CVD2.

    PTM databases

    PhosphoSiteiQ9CVD2.

    Expressioni

    Gene expression databases

    ArrayExpressiQ9CVD2.
    BgeeiQ9CVD2.
    CleanExiMM_ATXN3.
    GenevestigatoriQ9CVD2.

    Interactioni

    Subunit structurei

    Interacts with DNA repair proteins RAD23A and RAD23B By similarity. Interacts with STUB1/CHIP (when monoubiquitinated).By similarity1 Publication

    Protein-protein interaction databases

    BioGridi225755. 18 interactions.
    IntActiQ9CVD2. 2 interactions.
    MINTiMINT-2837817.

    Structurei

    3D structure databases

    ProteinModelPortaliQ9CVD2.
    SMRiQ9CVD2. Positions 1-171, 220-264.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini1 – 180180JosephinPROSITE-ProRule annotationAdd
    BLAST
    Repeati224 – 24320UIM 1Add
    BLAST
    Repeati244 – 26320UIM 2Add
    BLAST
    Repeati329 – 34820UIM 3Add
    BLAST

    Compositional bias

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Compositional biasi282 – 2854Poly-Arg
    Compositional biasi292 – 2976Poly-Gln

    Domaini

    The UIM domains bind ubiquitin and interact with various E3 ubiquitin-protein ligase, such as STUB1/CHIP. They are essential to limit the length of ubiquitin chains (PubMed:21855799).1 Publication

    Sequence similaritiesi

    Contains 1 Josephin domain.PROSITE-ProRule annotation
    Contains 3 UIM (ubiquitin-interacting motif) repeats.PROSITE-ProRule annotation

    Keywords - Domaini

    Repeat

    Phylogenomic databases

    eggNOGiNOG327234.
    GeneTreeiENSGT00390000001830.
    HOGENOMiHOG000006034.
    HOVERGENiHBG025648.
    InParanoidiQ9CVD2.
    KOiK11863.
    OMAiLQAAMNM.
    OrthoDBiEOG779NZ3.
    PhylomeDBiQ9CVD2.
    TreeFamiTF314228.

    Family and domain databases

    InterProiIPR006155. Josephin.
    IPR003903. Ubiquitin-int_motif.
    [Graphical view]
    PfamiPF02099. Josephin. 1 hit.
    PF02809. UIM. 3 hits.
    [Graphical view]
    PRINTSiPR01233. JOSEPHIN.
    SMARTiSM00726. UIM. 3 hits.
    [Graphical view]
    PROSITEiPS50957. JOSEPHIN. 1 hit.
    PS50330. UIM. 2 hits.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

    Q9CVD2-1 [UniParc]FASTAAdd to Basket

    « Hide

    MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERLRMAE    50
    GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ 100
    RLRIDPINER SFICNYKEHW FTVRKLGKQW FNLNSLLTGP ELISDTYLAL 150
    FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IKVQQMHRPK LIGEELAHLK 200
    EQSALKADLE RVLEAADGSG IFDEDEDDLQ RALAISRQEI DMEDEEADLR 250
    RAIQLSMQGS SRSMCENSPQ TSSPDLSSEE LRRRREAYFE KQQQQQQEVD 300
    RPGPLSYPRE RPTTSSGGRR SDQGGDAVSE EDMLRAAVTM SLETAKDNLK 350
    AERKK 355
    Length:355
    Mass (Da):40,533
    Last modified:February 28, 2003 - v2
    Checksum:iD148DD10EA481D5B
    GO

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AK008675 mRNA. Translation: BAB25825.3.
    AK030842 mRNA. Translation: BAC27155.1.
    CCDSiCCDS26115.1.
    RefSeqiNP_083981.2. NM_029705.3.
    UniGeneiMm.271914.

    Genome annotation databases

    EnsembliENSMUST00000021606; ENSMUSP00000021606; ENSMUSG00000021189.
    GeneIDi110616.
    KEGGimmu:110616.
    UCSCiuc007otv.2. mouse.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AK008675 mRNA. Translation: BAB25825.3 .
    AK030842 mRNA. Translation: BAC27155.1 .
    CCDSi CCDS26115.1.
    RefSeqi NP_083981.2. NM_029705.3.
    UniGenei Mm.271914.

    3D structure databases

    ProteinModelPortali Q9CVD2.
    SMRi Q9CVD2. Positions 1-171, 220-264.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 225755. 18 interactions.
    IntActi Q9CVD2. 2 interactions.
    MINTi MINT-2837817.

    Protein family/group databases

    MEROPSi C86.001.

    PTM databases

    PhosphoSitei Q9CVD2.

    Proteomic databases

    MaxQBi Q9CVD2.
    PaxDbi Q9CVD2.
    PRIDEi Q9CVD2.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000021606 ; ENSMUSP00000021606 ; ENSMUSG00000021189 .
    GeneIDi 110616.
    KEGGi mmu:110616.
    UCSCi uc007otv.2. mouse.

    Organism-specific databases

    CTDi 4287.
    MGIi MGI:1099442. Atxn3.

    Phylogenomic databases

    eggNOGi NOG327234.
    GeneTreei ENSGT00390000001830.
    HOGENOMi HOG000006034.
    HOVERGENi HBG025648.
    InParanoidi Q9CVD2.
    KOi K11863.
    OMAi LQAAMNM.
    OrthoDBi EOG779NZ3.
    PhylomeDBi Q9CVD2.
    TreeFami TF314228.

    Miscellaneous databases

    ChiTaRSi ATXN3. mouse.
    NextBioi 364339.
    PROi Q9CVD2.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi Q9CVD2.
    Bgeei Q9CVD2.
    CleanExi MM_ATXN3.
    Genevestigatori Q9CVD2.

    Family and domain databases

    InterProi IPR006155. Josephin.
    IPR003903. Ubiquitin-int_motif.
    [Graphical view ]
    Pfami PF02099. Josephin. 1 hit.
    PF02809. UIM. 3 hits.
    [Graphical view ]
    PRINTSi PR01233. JOSEPHIN.
    SMARTi SM00726. UIM. 3 hits.
    [Graphical view ]
    PROSITEi PS50957. JOSEPHIN. 1 hit.
    PS50330. UIM. 2 hits.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "The transcriptional landscape of the mammalian genome."
      Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
      , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
      Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Strain: NOD.
      Tissue: Stomach and Thymus.
    2. Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-219, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
      Tissue: Embryonic brain.
    3. Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-268 AND SER-273, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
      Tissue: Liver.
    4. "Structural modeling of ataxin-3 reveals distant homology to adaptins."
      Albrecht M., Hoffmann D., Evert B.O., Schmitt I., Wuellner U., Lengauer T.
      Proteins 50:355-370(2003) [PubMed] [Europe PMC] [Abstract]
      Cited for: 3D-STRUCTURE MODELING.
    5. Cited for: FUNCTION, INTERACTION WITH STUB1, UBIQUITINATION, MUTAGENESIS OF CYS-14.

    Entry informationi

    Entry nameiATX3_MOUSE
    AccessioniPrimary (citable) accession number: Q9CVD2
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: February 28, 2003
    Last sequence update: February 28, 2003
    Last modified: October 1, 2014
    This is version 106 of the entry and version 2 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. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3