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Protein

Ataxin-1

Gene

Atxn1

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript leveli

Functioni

Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression. Binds RNA in vitro. May be involved in RNA metabolism.By similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Repressor

Keywords - Biological processi

Transcription, Transcription regulation

Keywords - Ligandi

DNA-binding, RNA-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Ataxin-1
Alternative name(s):
Spinocerebellar ataxia type 1 protein homolog
Gene namesi
Name:Atxn1
Synonyms:Sca1
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome 17

Organism-specific databases

RGDi3624. Atxn1.

Subcellular locationi

  • Cytoplasm By similarity
  • Nucleus By similarity

  • Note: Colocalizes with USP7 in the nucleus.By similarity

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 789789Ataxin-1PRO_0000064753Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Cross-linki16 – 16Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity
Modified residuei81 – 811PhosphoserineBy similarity
Modified residuei87 – 871PhosphoserineBy similarity
Cross-linki193 – 193Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity
Modified residuei213 – 2131PhosphoserineBy similarity
Modified residuei217 – 2171PhosphothreonineBy similarity
Modified residuei228 – 2281PhosphoserineBy similarity
Cross-linki583 – 583Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity
Cross-linki670 – 670Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity
Cross-linki719 – 719Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity
Modified residuei749 – 7491PhosphoserineBy similarity

Post-translational modificationi

Ubiquitinated by UBE3A, leading to its degradation by the proteasome.By similarity
Phosphorylation at Ser-749 increases the pathogenicity of proteins with an expanded polyglutamine tract.By similarity
Sumoylation is dependent on nuclear localization and phosphorylation at Ser-749. It is reduced in the presence of an expanded polyglutamine tract.By similarity

Keywords - PTMi

Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

PaxDbiQ63540.

PTM databases

iPTMnetiQ63540.
PhosphoSiteiQ63540.

Expressioni

Gene expression databases

BgeeiENSRNOG00000016998.
GenevisibleiQ63540. RN.

Interactioni

Subunit structurei

Homooligomer (By similarity). Interacts with CIC (By similarity). Interacts with ANP32A, PQBP1, UBQLN4, ATXN1L, USP7 and ZNF804A. Directly interacts with RBPJ; this interaction is disrupted in the presence of Notch intracellular domain. Competes with ATXN1L for RBPJ-binding (By similarity).By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000023140.

Structurei

3D structure databases

ProteinModelPortaliQ63540.
SMRiQ63540. Positions 547-667.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini536 – 667132AXHPROSITE-ProRule annotationAdd
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni468 – 578111Self-associationBy similarityAdd
BLAST
Regioni512 – 789278Interaction with USP7By similarityAdd
BLAST
Regioni514 – 740227RNA-bindingBy similarityAdd
BLAST

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi768 – 7714Nuclear localization signalBy similarity

Domaini

The AXH domain is required for interaction with CIC.By similarity

Sequence similaritiesi

Belongs to the ATXN1 family.Curated
Contains 1 AXH domain.PROSITE-ProRule annotation

Phylogenomic databases

eggNOGiKOG4053. Eukaryota.
ENOG410XSNX. LUCA.
GeneTreeiENSGT00390000005939.
HOGENOMiHOG000034225.
HOVERGENiHBG004319.
InParanoidiQ63540.
OMAiHRSYALS.
OrthoDBiEOG091G0MXE.
PhylomeDBiQ63540.
TreeFamiTF350643.

Family and domain databases

InterProiIPR003652. Ataxin_AXH_dom.
IPR020997. Capicua_tscrpt_rep_mod.
[Graphical view]
PfamiPF12547. ATXN-1_C. 1 hit.
PF08517. AXH. 1 hit.
[Graphical view]
SMARTiSM00536. AXH. 1 hit.
[Graphical view]
SUPFAMiSSF102031. SSF102031. 1 hit.
PROSITEiPS51148. AXH. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Q63540-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKSNQERSNE CLPPKKREIP ATSRPSEEKA TALPSDNHCV EGVAWLPSTP
60 70 80 90 100
GSRGHGGGRH GPAGTSGEHG LQGMGLHKAL SAGLDYSPPS APRSVPTANT
110 120 130 140 150
LPTVYPPPQS GTPVSPVQYA HLSHTFQFIG SSQYSGPYAG FIPSQLISPP
160 170 180 190 200
GNPVTSAVAS AAGATTPSQR SQLEAYSTLL ANMGSLSQAP GHKVEPPPQQ
210 220 230 240 250
HLGRAAGLVN PGSPPPTQQN QYIHISSSPQ SSGRATSPPI PVHLHPHQTM
260 270 280 290 300
IPHTLTLGPS SQVVVQYSDA GGHFVPREST KKAESSRLQQ AMQAKEVLNG
310 320 330 340 350
EMEKSRRYGA SSSVELSLGK TSSKSVPHPY ESRHVVVHPS PADYSSRDTS
360 370 380 390 400
GVRGSVMVLP NSSTPSADLE TQQATHREAS PSTLNDKSGL HLGKPGHRSY
410 420 430 440 450
ALSPHTVIQT THSASEPLPV GLPATAFYAG AQPPVIGYLS SQQQAITYAG
460 470 480 490 500
GLPQHLVIPG TQPLLIPVGS PDMDTPGAAS AIVTSSPQFA AVPHTFVTTA
510 520 530 540 550
LPKSENFNPE ALVTQAAYPA MVQAQIHLPV VQSVASPAAA SPTLPPYFMK
560 570 580 590 600
GSIIQLANGE LKKVEDLKTE DFIQSAEISN DLKIDSSTVE RIEDSHSPGV
610 620 630 640 650
AVIQFAVGEH RAQVSVEVLV EYPFFVFGQG WSSCCPERTS QLFDLPCSKL
660 670 680 690 700
SVGDVCISLT LKNLKNGSVK KGQPVDPASA LLKHAKTDSL AGSRHRYAEQ
710 720 730 740 750
ENGINQGSAQ VLSENGELKF PEKIGLPAAP FLTKIEPSKP TATRKRRWSA
760 770 780
PETRKLEKSE DEPPLTLPKP SLIPQEVKIC IEGRSNVGK
Length:789
Mass (Da):83,501
Last modified:November 1, 1996 - v1
Checksum:iAFA042249BAC99FE
GO

Polymorphismi

The rat poly-Gln region is very limited in comparison to human ATXN1 and is not polymorphic.

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X91619 mRNA. Translation: CAA62822.1.
RefSeqiNP_036858.1. NM_012726.2.
UniGeneiRn.88438.

Genome annotation databases

EnsembliENSRNOT00000023140; ENSRNOP00000023140; ENSRNOG00000016998.
GeneIDi25049.
KEGGirno:25049.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X91619 mRNA. Translation: CAA62822.1.
RefSeqiNP_036858.1. NM_012726.2.
UniGeneiRn.88438.

3D structure databases

ProteinModelPortaliQ63540.
SMRiQ63540. Positions 547-667.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000023140.

PTM databases

iPTMnetiQ63540.
PhosphoSiteiQ63540.

Proteomic databases

PaxDbiQ63540.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSRNOT00000023140; ENSRNOP00000023140; ENSRNOG00000016998.
GeneIDi25049.
KEGGirno:25049.

Organism-specific databases

CTDi6310.
RGDi3624. Atxn1.

Phylogenomic databases

eggNOGiKOG4053. Eukaryota.
ENOG410XSNX. LUCA.
GeneTreeiENSGT00390000005939.
HOGENOMiHOG000034225.
HOVERGENiHBG004319.
InParanoidiQ63540.
OMAiHRSYALS.
OrthoDBiEOG091G0MXE.
PhylomeDBiQ63540.
TreeFamiTF350643.

Miscellaneous databases

PROiQ63540.

Gene expression databases

BgeeiENSRNOG00000016998.
GenevisibleiQ63540. RN.

Family and domain databases

InterProiIPR003652. Ataxin_AXH_dom.
IPR020997. Capicua_tscrpt_rep_mod.
[Graphical view]
PfamiPF12547. ATXN-1_C. 1 hit.
PF08517. AXH. 1 hit.
[Graphical view]
SMARTiSM00536. AXH. 1 hit.
[Graphical view]
SUPFAMiSSF102031. SSF102031. 1 hit.
PROSITEiPS51148. AXH. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiATX1_RAT
AccessioniPrimary (citable) accession number: Q63540
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 6, 2002
Last sequence update: November 1, 1996
Last modified: September 7, 2016
This is version 115 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

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.