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Index > Protein center > ATN1(Gene name) > Human
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  • ATN1 (Gene name),
  • Atrophin-1 (Protein name ),  ATN1_HUMAN from NCBI database.
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  • General Annotation
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  • Antigen Annotation
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  • 3D
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  • Predicted Eptitope
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  • Vaild Sequence
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  • Gene name:
    ATN1(D12S755E;DRPLA);
    Protein name:
    Atrophin-1;
    Alternative:
    Dentatorubral-pallidoluysian atrophy protein;
    Organism:
    Human (Homo sapiens). 
    General Annotation
    Sub Unit:
    Interacts with NR2E1; the interaction represses the transcriptional activity of NR2E1. Interacts (via its N-terminus) with FAT1 (via a C-terminus domain) (By similarity). Interacts with BAIAP2, WWP1, WWP2, WWP3 and RERE. Interacts (via its N-terminus) with MTG8; the interaction enhances transcriptional repression of MTG8.
    Function:
    Transcriptional corepressor. Recruits NR2E1 to repress transcription. Promotes vascular smooth cell (VSMC) migration and orientation (By similarity). Corepressor of MTG8 transcriptional repression. Has some intrinsic repression activity which is independent of the number of poly-Asn (polyQ) repeats.
    Subcellular Location:
    Nucleus Cytoplasm perinuclear region Cell junction Nucleus Shuttles between nucleus and cytoplasm. Colocalizes with FAT1 in the perinuclear area, at cell-cell junctions and leading edges of cells (By similarity). Colocalizes with MTG8 in discrete nuclear dots. Proteolytic fragment F1 appears to remain in nucleus. Fragment F2 is exported into the cytoplasm. Fragment F2 from mutant sequences with longer poly-Asn (polyQ) tracts are additionally located to the cytoplasmic membrane and to certain organelles.
    Protein Attributes:
    Sequence length:
    1190
    Sequence:
    50:
    MKTRQNKDSM | SMRSGRKKEA | PGPREELRSR | GRASPGGVST | SSSDGKAEKS | 
    100:
    RQTAKKARVE | EASTPKVNKQ | GRSEEISESE | SEETNAPKKT | KTEQELPRPQ | 
    150:
    SPSDLDSLDG | RSLNDDGSSD | PRDIDQDNRS | TSPSIYSPGS | VENDSDSSSG | 
    200:
    LSQGPARPYH | PPPLFPPSPQ | PPDSTPRQPE | ASFEPHPSVT | PTGYHAPMEP | 
    250:
    PTSRMFQAPP | GAPPPHPQLY | PGGTGGVLSG | PPMGPKGGGA | ASSVGGPNGG | 
    300:
    KQHPPPTTPI | SVSSSGASGA | PPTKPPTTPV | GGGNLPSAPP | PANFPHVTPN | 
    350:
    LPPPPALRPL | NNASASPPGL | GAQPLPGHLP | SPHAMGQGMG | GLPPGPEKGP | 
    400:
    TLAPSPHSLP | PASSSAPAPP | MRFPYSSSSS | SSAAASSSSS | SSSSSASPFP | 
    450:
    ASQALPSYPH | SFPPPTSLSV | SNQPPKYTQP | SLPSQAVWSQ | GPPPPPPYGR | 
    500:
    LLANSNAHPG | PFPPSTGAQS | TAHPPVSTHH | HHHQQQQQQQ | QQQQQQQQQQ | 
    550:
    QQHHGNSGPP | PPGAFPHPLE | GGSSHHAHPY | AMSPSLGSLR | PYPPGPAHLP | 
    600:
    PPHSQVSYSQ | AGPNGPPVSS | SSNSSSSTSQ | GSYPCSHPSP | SQGPQGAPYP | 
    650:
    FPPVPTVTTS | SATLSTVIAT | VASSPAGYKT | ASPPGPPPYG | KRAPSPGAYK | 
    700:
    TATPPGYKPG | SPPSFRTGTP | PGYRGTSPPA | GPGTFKPGSP | TVGPGPLPPA | 
    750:
    GPSGLPSLPP | PPAAPASGPP | LSATQIKQEP | AEEYETPESP | VPPARSPSPP | 
    800:
    PKVVDVPSHA | SQSARFNKHL | DRGFNSCARS | DLYFVPLEGS | KLAKKRADLV | 
    850:
    EKVRREAEQR | AREEKERERE | REREKERERE | KERELERSVK | LAQEGRAPVE | 
    900:
    CPSLGPVPHR | PPFEPGSAVA | TVPPYLGPDT | PALRTLSEYA | RPHVMSPGNR | 
    950:
    NHPFYVPLGA | VDPGLLGYNV | PALYSSDPAA | REREREARER | DLRDRLKPGF | 
    1000:
    EVKPSELEPL | HGVPGPGLDP | FPRHGGLALQ | PGPPGLHPFP | FHPSLGPLER | 
    1050:
    ERLALAAGPA | LRPDMSYAER | LAAERQHAER | VAALGNDPLA | RLQMLNVTPH | 
    1100:
    HHQHSHIHSH | LHLHQQDAIH | AASASVHPLI | DPLASGSHLT | RIPYPAGTLP | 
    1150:
    NPLLPHPLHE | NEVLRHQLFA | APYRDLPASL | SAPMSAAHQL | QAMHAQSAEL | 
    1190:
    QRLALEQQQW | LHAHHPLHSV | PLPAQEDYYS | HLKKESDKPL | 
    3D Structure:
    N/A
    Predicted Eptitope:
    Please Sign in.
    EIAab Sequence  Vaild Sequence:
    Please Sign in.
    Related Databases
    KEGG:
    String:
    UniGene:
    MIM:
    Pfam:
    Uniprot:
     
    FOR
    ELISA Kit for Human Atrophin-1
    ELISA Kit for Human Atrophin-1
    ELISA Kit for Human Atrophin-1
    CLIA Kit for Human Atrophin-1
    CLIA Kit for Human Atrophin-1
    CLIA Kit for Human Atrophin-1
    Polyclonal Antibody for Human Atrophin-1
    Polyclonal Antibody for Human Atrophin-1
    Polyclonal Antibody for Human Atrophin-1
    Monoclonal Antibody for Human Atrophin-1
    Monoclonal Antibody for Human Atrophin-1
    Monoclonal Antibody for Human Atrophin-1
    Protein for Human Atrophin-1
    Protein for Human Atrophin-1
    Protein for Human Atrophin-1

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    Linearity
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    References
    1. 1.
      "Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA)."
      Nagafuchi S. , Yanagisawa H. , Ohsaki E. , Shirayama T. , Tadokoro K. , Inoue T. , Yamada M.
      Nat. Genet.8:177-182(1994) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA];TISSUE SPECIFICITY;VARIANT 484-GLN--GLN-488 DEL
      tissue: Brain.
    2. 2.
      "Molecular cloning of a full-length cDNA for dentatorubral-pallidoluysian atrophy and regional expressions of the expanded alleles in the CNS."
      Onodera O. , Oyake M. , Takano H. , Ikeuchi T. , Igarashi S. , Tsuji S.
      Am. J. Hum. Genet.57:1050-1060(1995) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA];TISSUE SPECIFICITY;VARIANT 484-GLN--GLN-488 DEL
      tissue: Brain.
    3. 3.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA];TISSUE SPECIFICITY;VARIANTS ILE-339 AND 484-GLN--GLN-488 DEL
    4. 4.
      "Large-scale sequencing in human chromosome 12p13: experimental and computational gene structure determination."
      Ansari-Lari M.A. , Shen Y. , Muzny D.M. , Lee W. , Gibbs R.A.
      Genome Res.7:268-280(1997) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA];POLYMORPHISM OF POLY-GLN REGION
      tissue: Brain.
    5. 5.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1-76
    6. 6.
      "The Haw River syndrome: dentatorubropallidoluysian atrophy (DRPLA) in an African-American family."
      Burke J.R. , Wingfield M.S. , Lewis K.E. , Roses A.D. , Lee J.E. , Hulette C. , Pericak-Vance M.A. , Vance J.M.
      Nat. Genet.7:521-524(1994) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INVOLVEMENT IN HRS
    7. 7.
      "Dentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosis."
      Miyashita T. , Okamura-Oho Y. , Mito Y. , Nagafuchi S. , Yamada M.
      J. Biol. Chem.272:29238-29242(1997) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PROTEOLYTIC PROCESSING
    8. 8.
      "Atrophin-1, the DRPLA gene product, interacts with two families of WW domain-containing proteins."
      Wood J.D. , Yuan J. , Margolis R.L. , Colomer V. , Duan K. , Kushi J. , Kaminsky Z. , Kleiderlein J.J. Jr. , Sharp A.H. , Ross C.A.
      Mol. Cell. Neurosci.11:149-160(1998) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH WWP1; WWP2 AND WWP3
    9. 9.
      "Dentatorubral-pallidoluysian atrophy protein interacts through a proline-rich region near polyglutamine with the SH3 domain of an insulin receptor tyrosine kinase substrate."
      Okamura-Oho Y. , Miyashita T. , Ohmi K. , Yamada M.
      Hum. Mol. Genet.8:947-957(1999) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH BAIAP2;SUBCELLULAR LOCATION
    10. 10.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: CLEAVAGE AT ASP-109;FUNCTION;SUBCELLULAR LOCATION;MUTAGENESIS OF ASP-109
    11. 11.
      "Protein binding of a DRPLA family through arginine-glutamic acid dipeptide repeats is enhanced by extended polyglutamine."
      Yanagisawa H. , Bundo M. , Miyashita T. , Okamura-Oho Y. , Tadokoro K. , Tokunaga K. , Yamada M.
      Hum. Mol. Genet.9:1433-1442(2000) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH RERE
    12. 12.
      "Atrophin-1, the dentato-rubral and pallido-luysian atrophy gene product, interacts with ETO/MTG8 in the nuclear matrix and represses transcription."
      Wood J.D. , Nucifora F.C. Jr. , Duan K. , Zhang C. , Wang J. , Kim Y. , Schilling G. , Sacchi N. , Liu J.M. , Ross C.A.
      J. Cell Biol.150:939-948(2000) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH MTG8;SUBCELLULAR LOCATION;FUNCTION
    13. 13.
      "Dentatorubral-pallidoluysian atrophy protein is phosphorylated by c-Jun NH2-terminal kinase."
      Okamura-Oho Y. , Miyashita T. , Nagao K. , Shima S. , Ogata Y. , Katada T. , Nishina H. , Yamada M.
      Hum. Mol. Genet.12:1535-1542(2003) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PHOSPHORYLATION AT SER-739
    14. 14.
      "Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity."
      Nucifora F.C. Jr. , Ellerby L.M. , Wellington C.L. , Wood J.D. , Herring W.J. , Sawa A. , Hayden M.R. , Dawson V.L. , Dawson T.M. , Ross C.A.
      J. Biol. Chem.278:13047-13055(2003) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PROTEOLYTIC PROCESSING;NUCLEAR LOCALIZATION SIGNAL;NUCLEAR EXPORT SIGNAL;SUBCELLULAR LOCATION
    15. 15.
      "Global, in vivo, and site-specific phosphorylation dynamics in signaling networks."
      Olsen J.V. , Blagoev B. , Gnad F. , Macek B. , Kumar C. , Mortensen P. , Mann M.
      Cell127:635-648(2006) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
      tissue: Cervix carcinoma.
    16. 16.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-77; SER-79; SER-101; SER-103; SER-107; THR-653; SER-661; SER-746 AND SER-748;IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
      tissue: Cervix carcinoma.
    17. 17.
      "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]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH FAT1
    18. 18.
      "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
      Choudhary C. , Kumar C. , Gnad F. , Nielsen M.L. , Rehman M. , Walther T.C. , Olsen J.V. , Mann M.
      Science325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-641;IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
    19. 19.
      "Proteolytic processing regulates pathological accumulation in dentatorubral-pallidoluysian atrophy."
      Suzuki Y. , Nakayama K. , Hashimoto N. , Yazawa I.
      FEBS J.277:4873-4887(2010) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PROTEOLYTIC PROCESSING;SUBCELLULAR LOCATION
    20. 20.
      "Y65C missense mutation in the WW domain of the Golabi-Ito-Hall syndrome protein PQBP1 affects its binding activity and deregulates pre-mRNA splicing."
      Tapia V.E. , Nicolaescu E. , McDonald C.B. , Musi V. , Oka T. , Inayoshi Y. , Satteson A.C. , Mazack V. , Humbert J. , Gaffney C.J. , Beullens M. , Schwartz C.E. , Landgraf C. , Volkmer R. , Pastore A. , Farooq A. , Bollen M. , Sudol M.
      J. Biol. Chem.285:19391-19401(2010) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH PQBP1
    21. 21.
      "Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."
      Olsen J.V. , Vermeulen M. , Santamaria A. , Kumar C. , Miller M.L. , Jensen L.J. , Gnad F. , Cox J. , Jensen T.S. , Nigg E.A. , Brunak S. , Mann M.
      Sci. Signal.3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-34;IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
      tissue: Cervix carcinoma.
    22. 22.
      "System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation."
      Rigbolt K.T. , Prokhorova T.A. , Akimov V. , Henningsen J. , Johansen P.T. , Kratchmarova I. , Kassem M. , Mann M. , Olsen J.V. , Blagoev B.
      Sci. Signal.4:RS3-RS3(2011) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-661;IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
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