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Q9W689

- ATX3_CHICK

UniProt

Q9W689 - ATX3_CHICK

Protein

Ataxin-3

Gene

ATXN3

Organism
Gallus gallus (Chicken)
Status
Reviewed - Annotation score: 4 out of 5- Experimental evidence at transcript leveli
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    • History
      Entry version 86 (01 Oct 2014)
      Sequence version 1 (01 Nov 1999)
      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. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription By similarity.By similarity

    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 – 141NucleophilePROSITE-ProRule annotation
    Active sitei119 – 1191Proton acceptorPROSITE-ProRule annotation
    Active sitei134 – 1341PROSITE-ProRule annotation

    GO - Molecular functioni

    1. omega peptidase activity Source: InterPro
    2. ubiquitin protein ligase binding Source: UniProtKB
    3. ubiquitin-specific protease activity Source: UniProtKB
    4. ubiquitin thiolesterase activity Source: UniProtKB

    GO - Biological processi

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

    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:MDJ1, MJD
    OrganismiGallus gallus (Chicken)
    Taxonomic identifieri9031 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiTestudines + Archosauria groupArchosauriaDinosauriaSaurischiaTheropodaCoelurosauriaAvesNeognathaeGalliformesPhasianidaePhasianinaeGallus
    ProteomesiUP000000539: Chromosome 5

    Subcellular locationi

    Nucleus By similarity

    GO - Cellular componenti

    1. mitochondrial matrix Source: Ensembl
    2. mitochondrial membrane Source: Ensembl
    3. nuclear inclusion body Source: Ensembl

    Keywords - Cellular componenti

    Nucleus

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 363363Ataxin-3PRO_0000053834Add
    BLAST

    Expressioni

    Tissue specificityi

    Widely expressed.

    Structurei

    3D structure databases

    ProteinModelPortaliQ9W689.
    SMRiQ9W689. Positions 1-185.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini1 – 180180JosephinPROSITE-ProRule annotationAdd
    BLAST
    Repeati227 – 24620UIM 1Add
    BLAST
    Repeati247 – 26620UIM 2Add
    BLAST
    Repeati337 – 35620UIM 3Add
    BLAST

    Compositional bias

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Compositional biasi294 – 2974Poly-Arg
    Compositional biasi304 – 3118Poly-Gln

    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.
    InParanoidiQ9W689.
    KOiK11863.
    OMAiLQAAMNM.
    OrthoDBiEOG779NZ3.
    PhylomeDBiQ9W689.
    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. 3 hits.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

    Q9W689-1 [UniParc]FASTAAdd to Basket

    « Hide

    MESIFHERQE GSLCAQHCLN NLLQGEYFSP VELSSIAQQL DEEERMRMAE    50
    GGVSSEEYRT FLQQPSVNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ 100
    RLGIDPINEK SFICNYKEHW FTVRKLGKQW FNLNSLLMGP ELISDTYLAL 150
    FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQVQRPK LIGEETAQSR 200
    DQRLPRSDVD QAIEVSHPFD GTGMLDEDEE NFQRALALSR QEIDMEDEEA 250
    DLRRAIQLSM QGSRQSEFSN SLPQNASQPP HTSQTDSLSS EDLRRRRQAY 300
    FEKQQQQLQQ QDLTLNLHDK PTINSSTLEA DPGGDMSEED MLQAAMNMSL 350
    ESARNHLSTE EKK 363
    Length:363
    Mass (Da):41,608
    Last modified:November 1, 1999 - v1
    Checksum:i15C81537096DE8C4
    GO

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF085247 mRNA. Translation: AAD21923.1.
    RefSeqiNP_989688.1. NM_204357.1.
    UniGeneiGga.44490.

    Genome annotation databases

    EnsembliENSGALT00000017509; ENSGALP00000017488; ENSGALG00000010766.
    GeneIDi378424.
    KEGGigga:378424.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF085247 mRNA. Translation: AAD21923.1 .
    RefSeqi NP_989688.1. NM_204357.1.
    UniGenei Gga.44490.

    3D structure databases

    ProteinModelPortali Q9W689.
    SMRi Q9W689. Positions 1-185.
    ModBasei Search...
    MobiDBi Search...

    Protein family/group databases

    MEROPSi C86.001.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSGALT00000017509 ; ENSGALP00000017488 ; ENSGALG00000010766 .
    GeneIDi 378424.
    KEGGi gga:378424.

    Organism-specific databases

    CTDi 4287.

    Phylogenomic databases

    eggNOGi NOG327234.
    GeneTreei ENSGT00390000001830.
    HOGENOMi HOG000006034.
    HOVERGENi HBG025648.
    InParanoidi Q9W689.
    KOi K11863.
    OMAi LQAAMNM.
    OrthoDBi EOG779NZ3.
    PhylomeDBi Q9W689.
    TreeFami TF314228.

    Miscellaneous databases

    NextBioi 20813765.
    PROi Q9W689.

    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. 3 hits.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "Conserved domains and lack of evidence for polyglutamine length polymorphism in the chicken homolog of the Machado-Joseph disease gene product ataxin-3."
      Linhartova I., Repitz M., Draber P., Nemec M., Wiche G., Propst F.
      Biochim. Biophys. Acta 1444:299-305(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
      Strain: SPAFAS.
    2. "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.

    Entry informationi

    Entry nameiATX3_CHICK
    AccessioniPrimary (citable) accession number: Q9W689
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: February 28, 2003
    Last sequence update: November 1, 1999
    Last modified: October 1, 2014
    This is version 86 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. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3