Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Atrophin-1

Gene

Atn1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: 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.By similarity2 Publications

GO - Molecular functioni

  • protein domain specific binding Source: MGI
  • RNA polymerase II transcription coactivator activity Source: MGI
  • toxin receptor binding Source: MGI
  • transcription corepressor activity Source: MGI

GO - Biological processi

  • cell migration Source: UniProtKB
  • determination of adult lifespan Source: MGI
  • maintenance of cell polarity Source: UniProtKB
  • male gonad development Source: MGI
  • multicellular organism growth Source: MGI
  • negative regulation of transcription from RNA polymerase II promoter Source: MGI
  • neuron apoptotic process Source: MGI
  • post-embryonic development Source: MGI
  • response to food Source: MGI
  • spermatogenesis Source: MGI
  • toxin metabolic process Source: MGI
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
Proteomesi
  • UP000000589 Componenti: 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

  • cell junction Source: UniProtKB-SubCell
  • cytoplasm Source: MGI
  • nuclear matrix Source: MGI
  • nucleoplasm Source: MGI
  • nucleus Source: MGI
  • perinuclear region of cytoplasm Source: UniProtKB-SubCell
Complete GO annotation...

Keywords - Cellular componenti

Cell junction, Cytoplasm, Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003562531 – 1175Atrophin-1Add BLAST1175

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei34PhosphoserineBy similarity1
Modified residuei77PhosphoserineCombined sources1
Modified residuei79PhosphoserineCombined sources1
Modified residuei101PhosphoserineCombined sources1
Modified residuei103PhosphoserineBy similarity1
Modified residuei107PhosphoserineCombined sources1
Modified residuei617PhosphoserineBy similarity1
Modified residuei626N6-acetyllysineBy similarity1
Modified residuei638PhosphothreonineBy similarity1
Modified residuei646PhosphoserineBy similarity1
Modified residuei654PhosphothreonineBy similarity1
Modified residuei724Phosphoserine; by MAPK8By similarity1
Modified residuei731PhosphoserineBy similarity1
Modified residuei733PhosphoserineBy similarity1
Modified residuei881PhosphoserineBy similarity1
Modified residuei1100Asymmetric dimethylarginineCombined sources1

Post-translational modificationi

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

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei109 – 110CleavageSequence analysis2

Keywords - PTMi

Acetylation, Methylation, Phosphoprotein

Proteomic databases

EPDiO35126.
MaxQBiO35126.
PaxDbiO35126.
PeptideAtlasiO35126.
PRIDEiO35126.

PTM databases

iPTMnetiO35126.
PhosphoSitePlusiO35126.

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

BgeeiENSMUSG00000004263.
ExpressionAtlasiO35126. baseline and differential.
GenevisibleiO35126. MM.

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

GO - Molecular functioni

  • protein domain specific binding Source: MGI
  • toxin receptor binding Source: MGI

Protein-protein interaction databases

BioGridi199314. 2 interactors.
STRINGi10090.ENSMUSP00000085695.

Structurei

3D structure databases

ProteinModelPortaliO35126.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni503 – 553Involved in binding BAIAP2By similarityAdd BLAST51
Regioni864 – 879Required for interaction with FAT1By similarityAdd BLAST16

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi16 – 32Nuclear localization signalBy similarityAdd BLAST17
Motifi1018 – 1026Nuclear export signalBy similarity9

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi166 – 172Poly-Pro7
Compositional biasi304 – 307Poly-Pro4
Compositional biasi378 – 383Poly-Ser6
Compositional biasi387 – 396Poly-Ser10
Compositional biasi441 – 446Poly-Pro6
Compositional biasi477 – 480Poly-His4
Compositional biasi481 – 487Poly-Gln7
Compositional biasi495 – 498Poly-Pro4
Compositional biasi555 – 564Poly-Ser10
Compositional biasi694 – 697Poly-Pro4
Compositional biasi792 – 805Ala/Arg-richAdd BLAST14
Compositional biasi806 – 817Arg/Glu-rich (mixed charge)Add BLAST12
Compositional biasi915 – 924Arg/Glu-rich (mixed charge)10
Compositional biasi1035 – 1062His-richAdd BLAST28

Phylogenomic databases

eggNOGiKOG2133. Eukaryota.
ENOG410ZIND. LUCA.
GeneTreeiENSGT00580000081398.
HOGENOMiHOG000231091.
HOVERGENiHBG075369.
InParanoidiO35126.
KOiK05626.
OMAiGPEKGPT.
OrthoDBiEOG091G0O1H.
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

        10         20         30         40         50
MKTRQNKDSM SMRSGRKKEA PGPREELRSR GRASPGGVST SSSDGKAEKS
60 70 80 90 100
RQTAKKARIE EPSAPKASKQ GRSEEISESE SEETSAPKKT KTEQELPRPQ
110 120 130 140 150
SPSDLDSLDG RSINDDGSSD PRDIDQDNRS TSPSIYSPGS VENDSDSSSG
160 170 180 190 200
LSQGPARPYH PPPLFPPSPP PPDSTPRQPE SGFEPHPSVP PTGYHAPMEP
210 220 230 240 250
PTSRLFQGPP PGAPPTHPQL YPGNASGGVL SGPPMGPKGG AAASSVGAPS
260 270 280 290 300
GGKQHPPPTT PIPISSSGAS GAPPAKPPSA PVGGGSLPSA PPPASFPHVT
310 320 330 340 350
PNLPPPPALR PLNNASASPP GMGAQPIPGH LPSPHAMGQG MSGLPPGPEK
360 370 380 390 400
GPTLAPSPHP LPPASSSAPG PPMRYPYSSS SSSAAASSSS SSSSASQYPA
410 420 430 440 450
SQALPSYPHS FPPPTSMSVS NQPPKYTQPS LPSQAVWSQG PPPPPPYGRL
460 470 480 490 500
LANNNTHPGP FPPTGGQSTA HPAAPTHHHH QQQPQQQHHH GNSGPPPPGA
510 520 530 540 550
YPHPLESSNS HHAHPYNMSP SLGSLRPYPP GPAHLPPPHG QVSYNQAGPN
560 570 580 590 600
GPPVSSSNSS GSSSQASYSC SHPSSSQGPQ GASYPFPPVP PVTTSSATLS
610 620 630 640 650
TVIATVASSP AGYKTASPPG PPQYSKRAPS PGSYKTATPP GYKPGSPPSF
660 670 680 690 700
RTGTPPGYRG TSPPAGPGTF KPGSPTVGPG PLPPAGPSSL SSLPPPPAAP
710 720 730 740 750
TTGPPLTATQ IKQEPAEEYE PPESPVPPAR SPSPPPKVVD VPSHASQSAR
760 770 780 790 800
FNKHLDRGFN SCARSDLYFV PLEGSKLAKK RADLVEKVRR EAEQRAREEK
810 820 830 840 850
EREREREREK EREREKEREL ERSVKLAQEG RAPVECPSLG PVPHRPPFEP
860 870 880 890 900
GSAVATVPPY LGPDTPALRT LSEYARPHVM SPGNRNHPFY VPLGAVDPGL
910 920 930 940 950
LGYNVPALYS SDPAARERER EARERDLRDR LKPGFEVKPS ELEPLHGVPG
960 970 980 990 1000
PGLDPFPRHG GLALQPGPPG LHPFPFHPSL GPLERERLAL AAGPALRPDM
1010 1020 1030 1040 1050
SYAERLAAER QHAERVAALG NDPLARLQML NVTPHHHQHS HIHSHLHLHQ
1060 1070 1080 1090 1100
QDAIHAASAS VHPLIDPLAS GSHLTRIPYP AGTLPNPLLP HPLHENEVLR
1110 1120 1130 1140 1150
HQLFAAPYRD LPASLSAPMS AAHQLQAMHA QSAELQRLAL EQQQWLHAHH
1160 1170
PLHSVPLPAQ EDYYSHLKKE SDKPL
Length:1,175
Mass (Da):123,724
Last modified:January 1, 1998 - v1
Checksum:i8BEFFAB75DDC0F36
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti452A → G in BAA13450 (PubMed:9070948).Curated1
Sequence conflicti495P → A in BAA13450 (PubMed:9070948).Curated1
Sequence conflicti532P → A in BAA13450 (PubMed:9070948).Curated1
Sequence conflicti691S → L in BAA13450 (PubMed:9070948).Curated1
Sequence conflicti734P → A in BAA13450 (PubMed:9070948).Curated1
Sequence conflicti766D → T in BAA13450 (PubMed:9070948).Curated1
Sequence conflicti926D → N in BAA13450 (PubMed:9070948).Curated1

Polymorphismi

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

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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; ENSMUSG00000107478.
GeneIDi13498.
KEGGimmu:13498.
UCSCiuc009drq.1. mouse.

Keywords - Coding sequence diversityi

Polymorphism, Triplet repeat expansion

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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.

3D structure databases

ProteinModelPortaliO35126.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi199314. 2 interactors.
STRINGi10090.ENSMUSP00000085695.

PTM databases

iPTMnetiO35126.
PhosphoSitePlusiO35126.

Proteomic databases

EPDiO35126.
MaxQBiO35126.
PaxDbiO35126.
PeptideAtlasiO35126.
PRIDEiO35126.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000088357; ENSMUSP00000085695; ENSMUSG00000004263.
ENSMUST00000129411; ENSMUSP00000115407; ENSMUSG00000107478.
GeneIDi13498.
KEGGimmu:13498.
UCSCiuc009drq.1. mouse.

Organism-specific databases

CTDi1822.
MGIiMGI:104725. Atn1.

Phylogenomic databases

eggNOGiKOG2133. Eukaryota.
ENOG410ZIND. LUCA.
GeneTreeiENSGT00580000081398.
HOGENOMiHOG000231091.
HOVERGENiHBG075369.
InParanoidiO35126.
KOiK05626.
OMAiGPEKGPT.
OrthoDBiEOG091G0O1H.
PhylomeDBiO35126.
TreeFamiTF328554.

Miscellaneous databases

ChiTaRSiAtn1. mouse.
PROiO35126.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000004263.
ExpressionAtlasiO35126. baseline and differential.
GenevisibleiO35126. MM.

Family and domain databases

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

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: November 30, 2016
This is version 107 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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.