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Q9W689

- ATX3_CHICK

UniProt

Q9W689 - ATX3_CHICK

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Protein
Ataxin-3
Gene
ATXN3, MDJ1, MJD
Organism
Gallus gallus (Chicken)
Status
Reviewed - 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.

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 – 141Nucleophile By similarity
Active sitei119 – 1191Proton acceptor By similarity
Active sitei134 – 1341 By similarity

GO - Molecular functioni

  1. omega peptidase activity Source: InterPro
  2. ubiquitin protein ligase binding Source: UniProtKB
  3. ubiquitin thiolesterase activity Source: UniProtKB
  4. 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 cell-substrate adhesion Source: Ensembl
  8. regulation of transcription, DNA-templated Source: UniProtKB-KW
  9. 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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiTestudines + Archosauria groupArchosauriaDinosauriaSaurischiaTheropodaCoelurosauriaAvesNeognathaeGalliformesPhasianidaePhasianinaeGallus
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-3
PRO_0000053834Add
BLAST

Expressioni

Tissue specificityi

Widely expressed.

Structurei

3D structure databases

ProteinModelPortaliQ9W689.
SMRiQ9W689. Positions 1-185.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini1 – 180180Josephin
Add
BLAST
Repeati227 – 24620UIM 1
Add
BLAST
Repeati247 – 26620UIM 2
Add
BLAST
Repeati337 – 35620UIM 3
Add
BLAST

Compositional bias

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

Sequence similaritiesi

Contains 1 Josephin domain.

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiNOG327234.
GeneTreeiENSGT00390000001830.
HOGENOMiHOG000006034.
HOVERGENiHBG025648.
InParanoidiQ9W689.
KOiK11863.
OMAiLQAAMNM.
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

MESIFHERQE GSLCAQHCLN NLLQGEYFSP VELSSIAQQL DEEERMRMAE    50
GGVSSEEYRT FLQQPSVNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ 100
RLGIDPINEK SFICNYKEHW FTVRKLGKQW FNLNSLLMGP ELISDTYLAL 150
FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQVQRPK LIGEETAQSR 200
DQRLPRSDVD QAIEVSHPFD GTGMLDEDEE NFQRALALSR QEIDMEDEEA 250
DLRRAIQLSM QGSRQSEFSN SLPQNASQPP HTSQTDSLSS EDLRRRRQAY 300
FEKQQQQLQQ QDLTLNLHDK PTINSSTLEA DPGGDMSEED MLQAAMNMSL 350
ESARNHLSTE EKK 363
Length:363
Mass (Da):41,608
Last modified:November 1, 1999 - v1
Checksum:i15C81537096DE8C4
GO

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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:
EMBL
GenBank
DDBJ
Links Updated
AF085247 mRNA. Translation: AAD21923.1 .
RefSeqi NP_989688.1. NM_204357.1.
UniGenei Gga.44490.

3D structure databases

ProteinModelPortali Q9W689.
SMRi Q9W689. Positions 1-185.
ModBasei Search...
MobiDBi Search...

Protein family/group databases

MEROPSi C86.001.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSGALT00000017509 ; ENSGALP00000017488 ; ENSGALG00000010766 .
GeneIDi 378424.
KEGGi gga:378424.

Organism-specific databases

CTDi 4287.

Phylogenomic databases

eggNOGi NOG327234.
GeneTreei ENSGT00390000001830.
HOGENOMi HOG000006034.
HOVERGENi HBG025648.
InParanoidi Q9W689.
KOi K11863.
OMAi LQAAMNM.
OrthoDBi EOG779NZ3.
PhylomeDBi Q9W689.
TreeFami TF314228.

Miscellaneous databases

NextBioi 20813765.
PROi Q9W689.

Family and domain databases

InterProi IPR006155. Josephin.
IPR003903. Ubiquitin-int_motif.
[Graphical view ]
Pfami PF02099. Josephin. 1 hit.
PF02809. UIM. 3 hits.
[Graphical view ]
PRINTSi PR01233. JOSEPHIN.
SMARTi SM00726. UIM. 3 hits.
[Graphical view ]
PROSITEi PS50957. JOSEPHIN. 1 hit.
PS50330. UIM. 3 hits.
[Graphical view ]
ProtoNeti Search...

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: May 14, 2014
This is version 85 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

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