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Protein

Ataxin-3

Gene

ATXN3

Organism
Gallus gallus (Chicken)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at transcript leveli

Functioni

Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription (By similarity).By similarity

Catalytic activityi

Thiol-dependent hydrolysis of ester, thioester, amide, peptide and isopeptide bonds formed by the C-terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal).

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Active sitei14 – 141NucleophilePROSITE-ProRule annotation
Active sitei119 – 1191Proton acceptorPROSITE-ProRule annotation
Active sitei134 – 1341PROSITE-ProRule annotation

GO - Molecular functioni

  1. omega peptidase activity Source: InterPro
  2. ubiquitin protein ligase binding Source: UniProtKB
  3. ubiquitin-specific protease activity Source: UniProtKB

GO - Biological processi

  1. cellular response to heat Source: Ensembl
  2. cellular response to misfolded protein Source: UniProtKB
  3. exploration behavior Source: Ensembl
  4. misfolded or incompletely synthesized protein catabolic process Source: UniProtKB
  5. monoubiquitinated protein deubiquitination Source: UniProtKB
  6. proteasome-mediated ubiquitin-dependent protein catabolic process Source: UniProtKB
  7. regulation of transcription, DNA-templated Source: UniProtKB-KW
  8. transcription, DNA-templated Source: UniProtKB-KW
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Protease, Thiol protease

Keywords - Biological processi

Transcription, Transcription regulation, Ubl conjugation pathway

Protein family/group databases

MEROPSiC86.001.

Names & Taxonomyi

Protein namesi
Recommended name:
Ataxin-3 (EC:3.4.19.12)
Alternative name(s):
Machado-Joseph disease protein 1 homolog
Gene namesi
Name:ATXN3
Synonyms:MDJ1, MJD
OrganismiGallus gallus (Chicken)
Taxonomic identifieri9031 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiArchelosauriaArchosauriaDinosauriaSaurischiaTheropodaCoelurosauriaAvesNeognathaeGalloanseraeGalliformesPhasianidaePhasianinaeGallus
ProteomesiUP000000539: Chromosome 5

Subcellular locationi

Nucleus By similarity

GO - Cellular componenti

  1. mitochondrial matrix Source: Ensembl
  2. mitochondrial membrane Source: Ensembl
  3. nuclear inclusion body Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 363363Ataxin-3PRO_0000053834Add
BLAST

Expressioni

Tissue specificityi

Widely expressed.

Structurei

3D structure databases

ProteinModelPortaliQ9W689.
SMRiQ9W689. Positions 1-185.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini1 – 180180JosephinPROSITE-ProRule annotationAdd
BLAST
Domaini227 – 24620UIM 1PROSITE-ProRule annotationAdd
BLAST
Domaini247 – 26620UIM 2PROSITE-ProRule annotationAdd
BLAST
Domaini337 – 35620UIM 3PROSITE-ProRule annotationAdd
BLAST

Compositional bias

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Compositional biasi294 – 2974Poly-Arg
Compositional biasi304 – 3118Poly-Gln

Sequence similaritiesi

Contains 1 Josephin domain.PROSITE-ProRule annotation
Contains 3 UIM (ubiquitin-interacting motif) domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiNOG327234.
GeneTreeiENSGT00390000001830.
HOGENOMiHOG000006034.
HOVERGENiHBG025648.
InParanoidiQ9W689.
KOiK11863.
OMAiEDEENFQ.
OrthoDBiEOG779NZ3.
PhylomeDBiQ9W689.
TreeFamiTF314228.

Family and domain databases

InterProiIPR006155. Josephin.
IPR003903. Ubiquitin-int_motif.
[Graphical view]
PfamiPF02099. Josephin. 1 hit.
PF02809. UIM. 3 hits.
[Graphical view]
PRINTSiPR01233. JOSEPHIN.
SMARTiSM00726. UIM. 3 hits.
[Graphical view]
PROSITEiPS50957. JOSEPHIN. 1 hit.
PS50330. UIM. 3 hits.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Q9W689-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MESIFHERQE GSLCAQHCLN NLLQGEYFSP VELSSIAQQL DEEERMRMAE
60 70 80 90 100
GGVSSEEYRT FLQQPSVNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ
110 120 130 140 150
RLGIDPINEK SFICNYKEHW FTVRKLGKQW FNLNSLLMGP ELISDTYLAL
160 170 180 190 200
FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQVQRPK LIGEETAQSR
210 220 230 240 250
DQRLPRSDVD QAIEVSHPFD GTGMLDEDEE NFQRALALSR QEIDMEDEEA
260 270 280 290 300
DLRRAIQLSM QGSRQSEFSN SLPQNASQPP HTSQTDSLSS EDLRRRRQAY
310 320 330 340 350
FEKQQQQLQQ QDLTLNLHDK PTINSSTLEA DPGGDMSEED MLQAAMNMSL
360
ESARNHLSTE EKK
Length:363
Mass (Da):41,608
Last modified:November 1, 1999 - v1
Checksum:i15C81537096DE8C4
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF085247 mRNA. Translation: AAD21923.1.
RefSeqiNP_989688.1. NM_204357.1.
UniGeneiGga.44490.

Genome annotation databases

EnsembliENSGALT00000017509; ENSGALP00000017488; ENSGALG00000010766.
GeneIDi378424.
KEGGigga:378424.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF085247 mRNA. Translation: AAD21923.1.
RefSeqiNP_989688.1. NM_204357.1.
UniGeneiGga.44490.

3D structure databases

ProteinModelPortaliQ9W689.
SMRiQ9W689. Positions 1-185.
ModBaseiSearch...
MobiDBiSearch...

Protein family/group databases

MEROPSiC86.001.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSGALT00000017509; ENSGALP00000017488; ENSGALG00000010766.
GeneIDi378424.
KEGGigga:378424.

Organism-specific databases

CTDi4287.

Phylogenomic databases

eggNOGiNOG327234.
GeneTreeiENSGT00390000001830.
HOGENOMiHOG000006034.
HOVERGENiHBG025648.
InParanoidiQ9W689.
KOiK11863.
OMAiEDEENFQ.
OrthoDBiEOG779NZ3.
PhylomeDBiQ9W689.
TreeFamiTF314228.

Miscellaneous databases

NextBioi20813765.
PROiQ9W689.

Family and domain databases

InterProiIPR006155. Josephin.
IPR003903. Ubiquitin-int_motif.
[Graphical view]
PfamiPF02099. Josephin. 1 hit.
PF02809. UIM. 3 hits.
[Graphical view]
PRINTSiPR01233. JOSEPHIN.
SMARTiSM00726. UIM. 3 hits.
[Graphical view]
PROSITEiPS50957. JOSEPHIN. 1 hit.
PS50330. UIM. 3 hits.
[Graphical view]
ProtoNetiSearch...

Publicationsi

  1. "Conserved domains and lack of evidence for polyglutamine length polymorphism in the chicken homolog of the Machado-Joseph disease gene product ataxin-3."
    Linhartova I., Repitz M., Draber P., Nemec M., Wiche G., Propst F.
    Biochim. Biophys. Acta 1444:299-305(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: SPAFAS.
  2. "Structural modeling of ataxin-3 reveals distant homology to adaptins."
    Albrecht M., Hoffmann D., Evert B.O., Schmitt I., Wuellner U., Lengauer T.
    Proteins 50:355-370(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: 3D-STRUCTURE MODELING.

Entry informationi

Entry nameiATX3_CHICK
AccessioniPrimary (citable) accession number: Q9W689
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 28, 2003
Last sequence update: November 1, 1999
Last modified: March 4, 2015
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

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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 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.