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O35126

- ATN1_MOUSE

UniProt

O35126 - ATN1_MOUSE

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Protein

Atrophin-1

Gene
Atn1, Drpla
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

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.2 Publications

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sitei109 – 1102Cleavage Reviewed prediction

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
Complete GO annotation...

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

Nucleus. Cytoplasmperinuclear region. 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.

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
Complete GO annotation...

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 – 341Phosphoserine By similarity
Modified residuei77 – 771Phosphoserine By similarity
Modified residuei79 – 791Phosphoserine By similarity
Modified residuei101 – 1011Phosphoserine By similarity
Modified residuei103 – 1031Phosphoserine By similarity
Modified residuei107 – 1071Phosphoserine By similarity
Modified residuei626 – 6261N6-acetyllysine By similarity
Modified residuei638 – 6381Phosphothreonine By similarity
Modified residuei646 – 6461Phosphoserine By similarity
Modified residuei724 – 7241Phosphoserine; by MAPK8 By similarity
Modified residuei731 – 7311Phosphoserine By similarity
Modified residuei733 – 7331Phosphoserine By 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).2 Publications

Protein-protein interaction databases

BioGridi199314. 2 interactions.

Structurei

3D structure databases

ProteinModelPortaliO35126.

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni503 – 55351Involved in binding BAIAP2 By similarityAdd
BLAST
Regioni864 – 87916Required for interaction with FAT1 By similarityAdd
BLAST

Motif

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

Polymorphismi

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

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti452 – 4521A → G in BAA13450. 1 Publication
Sequence conflicti495 – 4951P → A in BAA13450. 1 Publication
Sequence conflicti532 – 5321P → A in BAA13450. 1 Publication
Sequence conflicti691 – 6911S → L in BAA13450. 1 Publication
Sequence conflicti734 – 7341P → A in BAA13450. 1 Publication
Sequence conflicti766 – 7661D → T in BAA13450. 1 Publication
Sequence conflicti926 – 9261D → N in BAA13450. 1 Publication

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

« Hide 'large scale' publications
  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: September 3, 2014
This is version 89 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

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