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O35126

- ATN1_MOUSE

UniProt

O35126 - ATN1_MOUSE

Protein

Atrophin-1

Gene

Atn1

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 90 (01 Oct 2014)
      Sequence version 1 (01 Jan 1998)
      Previous versions | rss
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    Functioni

    Transcriptional corepressor. Corepressor of MTG8 transcriptional repression. Has some intrinsic repression activity which is independent of the number of the poly-Q repeats By similarity. Recruits NR2E1 to repress transcription. Promotes vascular smooth cell (VSMC) migration and orientation.By similarity2 Publications

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sitei109 – 1102CleavageSequence Analysis

    GO - Molecular functioni

    1. protein binding Source: MGI
    2. toxin receptor binding Source: MGI
    3. transcription corepressor activity Source: MGI

    GO - Biological processi

    1. cell migration Source: UniProtKB
    2. maintenance of cell polarity Source: UniProtKB
    3. negative regulation of transcription from RNA polymerase II promoter Source: MGI
    4. neuron apoptotic process Source: Ensembl
    5. toxin metabolic process Source: MGI
    6. transcription, DNA-templated Source: UniProtKB-KW

    Keywords - Biological processi

    Transcription, Transcription regulation

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Atrophin-1
    Alternative name(s):
    Dentatorubral-pallidoluysian atrophy protein homolog
    Gene namesi
    Name:Atn1
    Synonyms:Drpla
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
    ProteomesiUP000000589: Chromosome 6

    Organism-specific databases

    MGIiMGI:104725. Atn1.

    Subcellular locationi

    Cytoplasmperinuclear region By similarity. Cell junction By similarity. Nucleus By similarity
    Note: Shuttles between nucleus and cytoplasm. Colocalizes with FAT1 in the perinuclear area, at cell-cell junctions and leading edges of cells. Colocalizes with MTG8 in discrete nuclear dots By similarity.By similarity

    GO - Cellular componenti

    1. cell junction Source: UniProtKB-SubCell
    2. cytoplasm Source: MGI
    3. nuclear matrix Source: Ensembl
    4. nucleus Source: MGI
    5. perinuclear region of cytoplasm Source: UniProtKB-SubCell

    Keywords - Cellular componenti

    Cell junction, Cytoplasm, Nucleus

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 11751175Atrophin-1PRO_0000356253Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei34 – 341PhosphoserineBy similarity
    Modified residuei77 – 771PhosphoserineBy similarity
    Modified residuei79 – 791PhosphoserineBy similarity
    Modified residuei101 – 1011PhosphoserineBy similarity
    Modified residuei103 – 1031PhosphoserineBy similarity
    Modified residuei107 – 1071PhosphoserineBy similarity
    Modified residuei626 – 6261N6-acetyllysineBy similarity
    Modified residuei638 – 6381PhosphothreonineBy similarity
    Modified residuei646 – 6461PhosphoserineBy similarity
    Modified residuei724 – 7241Phosphoserine; by MAPK8By similarity
    Modified residuei731 – 7311PhosphoserineBy similarity
    Modified residuei733 – 7331PhosphoserineBy similarity

    Post-translational modificationi

    Phosphorylated in vitro by MAPK8/JNK1 on Ser-724.By similarity

    Keywords - PTMi

    Acetylation, Phosphoprotein

    Proteomic databases

    PaxDbiO35126.
    PRIDEiO35126.

    PTM databases

    PhosphoSiteiO35126.

    Expressioni

    Tissue specificityi

    Widely expressed. Most abundant in the brain.1 Publication

    Developmental stagei

    Expressed as early as 5 days and thereafter shows little variation throughout 17 days.1 Publication

    Inductioni

    In vascular smooth muscle, induced by angiotensin II, FGF; PGF and IL1B.1 Publication

    Gene expression databases

    ArrayExpressiO35126.
    BgeeiO35126.
    GenevestigatoriO35126.

    Interactioni

    Subunit structurei

    Interacts with BAIAP2, WWP1, WWP2, WWP3 and RERE. Interacts (via its N-terminus) with MTG8; the interaction enhances transcriptional repression of MTG8. Interacts with PQBP1 By similarity. Interacts with NR2E1; the interaction represses the transcriptional activity of NR2E1. Interacts with FAT1 (via a C-terminal domain).By similarity2 Publications

    Protein-protein interaction databases

    BioGridi199314. 2 interactions.

    Structurei

    3D structure databases

    ProteinModelPortaliO35126.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Region

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Regioni503 – 55351Involved in binding BAIAP2By similarityAdd
    BLAST
    Regioni864 – 87916Required for interaction with FAT1By similarityAdd
    BLAST

    Motif

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Motifi16 – 3217Nuclear localization signalBy similarityAdd
    BLAST
    Motifi1018 – 10269Nuclear export signalBy similarity

    Compositional bias

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Compositional biasi166 – 1727Poly-Pro
    Compositional biasi304 – 3074Poly-Pro
    Compositional biasi378 – 3836Poly-Ser
    Compositional biasi387 – 39610Poly-Ser
    Compositional biasi441 – 4466Poly-Pro
    Compositional biasi477 – 4804Poly-His
    Compositional biasi481 – 4877Poly-Gln
    Compositional biasi495 – 4984Poly-Pro
    Compositional biasi555 – 56410Poly-Ser
    Compositional biasi694 – 6974Poly-Pro
    Compositional biasi792 – 80514Ala/Arg-richAdd
    BLAST
    Compositional biasi806 – 81712Arg/Glu-rich (mixed charge)Add
    BLAST
    Compositional biasi915 – 92410Arg/Glu-rich (mixed charge)
    Compositional biasi1035 – 106228His-richAdd
    BLAST

    Phylogenomic databases

    eggNOGiNOG331121.
    GeneTreeiENSGT00580000081398.
    HOGENOMiHOG000231091.
    HOVERGENiHBG075369.
    InParanoidiO35126.
    KOiK05626.
    OMAiGPEKGPT.
    OrthoDBiEOG7D59MN.
    PhylomeDBiO35126.
    TreeFamiTF328554.

    Family and domain databases

    InterProiIPR017993. Atrophin-1.
    IPR002951. Atrophin-like.
    [Graphical view]
    PfamiPF03154. Atrophin-1. 2 hits.
    [Graphical view]
    PRINTSiPR01222. ATROPHIN.

    Sequencei

    Sequence statusi: Complete.

    O35126-1 [UniParc]FASTAAdd to Basket

    « Hide

    MKTRQNKDSM SMRSGRKKEA PGPREELRSR GRASPGGVST SSSDGKAEKS     50
    RQTAKKARIE EPSAPKASKQ GRSEEISESE SEETSAPKKT KTEQELPRPQ 100
    SPSDLDSLDG RSINDDGSSD PRDIDQDNRS TSPSIYSPGS VENDSDSSSG 150
    LSQGPARPYH PPPLFPPSPP PPDSTPRQPE SGFEPHPSVP PTGYHAPMEP 200
    PTSRLFQGPP PGAPPTHPQL YPGNASGGVL SGPPMGPKGG AAASSVGAPS 250
    GGKQHPPPTT PIPISSSGAS GAPPAKPPSA PVGGGSLPSA PPPASFPHVT 300
    PNLPPPPALR PLNNASASPP GMGAQPIPGH LPSPHAMGQG MSGLPPGPEK 350
    GPTLAPSPHP LPPASSSAPG PPMRYPYSSS SSSAAASSSS SSSSASQYPA 400
    SQALPSYPHS FPPPTSMSVS NQPPKYTQPS LPSQAVWSQG PPPPPPYGRL 450
    LANNNTHPGP FPPTGGQSTA HPAAPTHHHH QQQPQQQHHH GNSGPPPPGA 500
    YPHPLESSNS HHAHPYNMSP SLGSLRPYPP GPAHLPPPHG QVSYNQAGPN 550
    GPPVSSSNSS GSSSQASYSC SHPSSSQGPQ GASYPFPPVP PVTTSSATLS 600
    TVIATVASSP AGYKTASPPG PPQYSKRAPS PGSYKTATPP GYKPGSPPSF 650
    RTGTPPGYRG TSPPAGPGTF KPGSPTVGPG PLPPAGPSSL SSLPPPPAAP 700
    TTGPPLTATQ IKQEPAEEYE PPESPVPPAR SPSPPPKVVD VPSHASQSAR 750
    FNKHLDRGFN SCARSDLYFV PLEGSKLAKK RADLVEKVRR EAEQRAREEK 800
    EREREREREK EREREKEREL ERSVKLAQEG RAPVECPSLG PVPHRPPFEP 850
    GSAVATVPPY LGPDTPALRT LSEYARPHVM SPGNRNHPFY VPLGAVDPGL 900
    LGYNVPALYS SDPAARERER EARERDLRDR LKPGFEVKPS ELEPLHGVPG 950
    PGLDPFPRHG GLALQPGPPG LHPFPFHPSL GPLERERLAL AAGPALRPDM 1000
    SYAERLAAER QHAERVAALG NDPLARLQML NVTPHHHQHS HIHSHLHLHQ 1050
    QDAIHAASAS VHPLIDPLAS GSHLTRIPYP AGTLPNPLLP HPLHENEVLR 1100
    HQLFAAPYRD LPASLSAPMS AAHQLQAMHA QSAELQRLAL EQQQWLHAHH 1150
    PLHSVPLPAQ EDYYSHLKKE SDKPL 1175
    Length:1,175
    Mass (Da):123,724
    Last modified:January 1, 1998 - v1
    Checksum:i8BEFFAB75DDC0F36
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti452 – 4521A → G in BAA13450. (PubMed:9070948)Curated
    Sequence conflicti495 – 4951P → A in BAA13450. (PubMed:9070948)Curated
    Sequence conflicti532 – 5321P → A in BAA13450. (PubMed:9070948)Curated
    Sequence conflicti691 – 6911S → L in BAA13450. (PubMed:9070948)Curated
    Sequence conflicti734 – 7341P → A in BAA13450. (PubMed:9070948)Curated
    Sequence conflicti766 – 7661D → T in BAA13450. (PubMed:9070948)Curated
    Sequence conflicti926 – 9261D → N in BAA13450. (PubMed:9070948)Curated

    Polymorphismi

    The poly-Gln region of Atn1 is polymorphic (3 to 8 repeats).

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    D87744 mRNA. Translation: BAA13450.1.
    AC002397 Genomic DNA. Translation: AAC36003.1.
    CH466523 Genomic DNA. Translation: EDK99754.1.
    CH466523 Genomic DNA. Translation: EDK99755.1.
    BC050920 mRNA. Translation: AAH50920.2.
    BC053051 mRNA. Translation: AAH53051.1.
    CCDSiCCDS20526.1.
    RefSeqiNP_031907.2. NM_007881.4.
    UniGeneiMm.333380.

    Genome annotation databases

    EnsembliENSMUST00000088357; ENSMUSP00000085695; ENSMUSG00000004263.
    ENSMUST00000129411; ENSMUSP00000115407; ENSMUSG00000004263.
    GeneIDi13498.
    KEGGimmu:13498.
    UCSCiuc009drq.1. mouse.

    Keywords - Coding sequence diversityi

    Polymorphism, Triplet repeat expansion

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    D87744 mRNA. Translation: BAA13450.1 .
    AC002397 Genomic DNA. Translation: AAC36003.1 .
    CH466523 Genomic DNA. Translation: EDK99754.1 .
    CH466523 Genomic DNA. Translation: EDK99755.1 .
    BC050920 mRNA. Translation: AAH50920.2 .
    BC053051 mRNA. Translation: AAH53051.1 .
    CCDSi CCDS20526.1.
    RefSeqi NP_031907.2. NM_007881.4.
    UniGenei Mm.333380.

    3D structure databases

    ProteinModelPortali O35126.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 199314. 2 interactions.

    PTM databases

    PhosphoSitei O35126.

    Proteomic databases

    PaxDbi O35126.
    PRIDEi O35126.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000088357 ; ENSMUSP00000085695 ; ENSMUSG00000004263 .
    ENSMUST00000129411 ; ENSMUSP00000115407 ; ENSMUSG00000004263 .
    GeneIDi 13498.
    KEGGi mmu:13498.
    UCSCi uc009drq.1. mouse.

    Organism-specific databases

    CTDi 1822.
    MGIi MGI:104725. Atn1.

    Phylogenomic databases

    eggNOGi NOG331121.
    GeneTreei ENSGT00580000081398.
    HOGENOMi HOG000231091.
    HOVERGENi HBG075369.
    InParanoidi O35126.
    KOi K05626.
    OMAi GPEKGPT.
    OrthoDBi EOG7D59MN.
    PhylomeDBi O35126.
    TreeFami TF328554.

    Miscellaneous databases

    ChiTaRSi ATN1. mouse.
    NextBioi 284033.
    PROi O35126.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi O35126.
    Bgeei O35126.
    Genevestigatori O35126.

    Family and domain databases

    InterProi IPR017993. Atrophin-1.
    IPR002951. Atrophin-like.
    [Graphical view ]
    Pfami PF03154. Atrophin-1. 2 hits.
    [Graphical view ]
    PRINTSi PR01222. ATROPHIN.
    ProtoNeti Search...

    Publicationsi

    1. "Molecular cloning of murine homologue dentatorubral-pallidoluysian atrophy (DRPLA) cDNA: strong conservation of a polymorphic CAG repeat in the murine gene."
      Oyake M., Onodera O., Shiroishi T., Takano H., Takahashi Y., Komonami R., Moriwaki K., Ikeuchi T., Igarashi S., Tanaka H., Tsuji S.
      Genomics 40:205-207(1997) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], TISSUE SPECIFICITY, DEVELOPMENTAL STAGE, POLYMORPHISM OF POLY-GLN REGION.
    2. "Comparative sequence analysis of a gene-rich cluster at human chromosome 12p13 and its syntenic region in mouse chromosome 6."
      Ansari-Lari M.A., Oeltjen J.C., Schwartz S., Zhang Z., Muzny D.M., Lu J., Gorrell J.H., Chinault A.C., Belmont J.W., Miller W., Gibbs R.A.
      Genome Res. 8:29-40(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
    3. Mural R.J., Adams M.D., Myers E.W., Smith H.O., Venter J.C.
      Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    4. "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].
      Strain: C57BL/6.
      Tissue: Brain.
    5. "Nuclear receptor TLX prevents retinal dystrophy and recruits the corepressor atrophin1."
      Zhang C.L., Zou Y., Yu R.T., Gage F.H., Evans R.M.
      Genes Dev. 20:1308-1320(2006) [PubMed] [Europe PMC] [Abstract]
      Cited for: INTERACTION WITH NR2E1, FUNCTION.
    6. "Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice."
      Sato T., Miura M., Yamada M., Yoshida T., Wood J.D., Yazawa I., Masuda M., Suzuki T., Shin R.M., Yau H.J., Liu F.C., Shimohata T., Onodera O., Ross C.A., Katsuki M., Takahashi H., Kano M., Aosaki T., Tsuji S.
      Hum. Mol. Genet. 18:723-736(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: CHARACTERISTICS OF THE MOUSE MODEL OF DRPLA.
    7. "Atrophin proteins interact with the Fat1 cadherin and regulate migration and orientation in vascular smooth muscle cells."
      Hou R., Sibinga N.E.
      J. Biol. Chem. 284:6955-6965(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: INTERACTION WITH FAT1, INDUCTION, FUNCTION.

    Entry informationi

    Entry nameiATN1_MOUSE
    AccessioniPrimary (citable) accession number: O35126
    Secondary accession number(s): P70200, Q80YQ0
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: December 16, 2008
    Last sequence update: January 1, 1998
    Last modified: October 1, 2014
    This is version 90 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Miscellaneous

    The mouse model of DRPLA with 129 CAG repeats (Q129) exhibited severe neurological defects similar to those of juvenile-onset DRPLA patients including age-dependent and region-specific presynaptic dysfunction in the globus pallidus and cerebellum. Progressive shrinkage of distal dendrites of Purkinje cells and decreased currents through alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid and gamma-aminobutyrate type A receptors in CA1 neurons was observed. There is progressive brain atrophy.

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

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

    External Data

    Dasty 3