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Protein

Ataxin-3

Gene

Atxn3

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

Functioni

Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:17696782). Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (By similarity). Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension (By similarity). Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription (By similarity). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (By similarity).By similarity1 Publication

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).1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei14NucleophileBy similarity1
Active sitei119Proton acceptorPROSITE-ProRule annotation1
Active sitei134PROSITE-ProRule annotation1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionHydrolase, Protease, Thiol protease
Biological processTranscription, Transcription regulation, Ubl conjugation pathway

Enzyme and pathway databases

ReactomeiR-RNO-5689877. Josephin domain DUBs.

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:Mjd, Sca3
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome 6

Organism-specific databases

RGDi621567. Atxn3.

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Nucleus

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi14C → A or S: Loss of enzyme activity. 1 Publication1
Mutagenesisi119H → A: Loss of enzyme activity. 1 Publication1
Mutagenesisi134N → A: Loss of enzyme activity. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000538331 – 355Ataxin-3Add BLAST355

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki1Peptide (Met-Gly) (interchain with G-Cter in ubiquitin)By similarity
Cross-linki200Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)By similarity
Modified residuei268PhosphoserineBy similarity1
Modified residuei272PhosphoserineBy similarity1
Modified residuei273PhosphoserineBy similarity1
Modified residuei321PhosphoserineBy similarity1

Post-translational modificationi

Monoubiquitinated by UBE2W, possibly leading to activate the deubiquitinating enzyme activity (By similarity).By similarity

Keywords - PTMi

Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

PaxDbiO35815.
PRIDEiO35815.

PTM databases

iPTMnetiO35815.
PhosphoSitePlusiO35815.

Expressioni

Tissue specificityi

Ubiquitously expressed (PubMed:10732811).1 Publication

Gene expression databases

BgeeiENSRNOG00000005470.
GenevisibleiO35815. RN.

Interactioni

Subunit structurei

Interacts with DNA repair proteins RAD23A and RAD23B (By similarity). Interacts with STUB1/CHIP (when monoubiquitinated) (By similarity). Interacts with BECN1 (via its poly-Gln domain) (By similarity).By similarity

GO - Molecular functioni

Protein-protein interaction databases

BioGridi248780. 2 interactors.
STRINGi10116.ENSRNOP00000007505.

Structurei

3D structure databases

ProteinModelPortaliO35815.
SMRiO35815.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini1 – 180JosephinPROSITE-ProRule annotationAdd BLAST180
Domaini224 – 243UIM 1PROSITE-ProRule annotationAdd BLAST20
Domaini244 – 263UIM 2PROSITE-ProRule annotationAdd BLAST20
Domaini329 – 348UIM 3PROSITE-ProRule annotationAdd BLAST20

Domaini

The UIM domains bind ubiquitin and interact with various E3 ubiquitin-protein ligase, such as STUB1/CHIP (By similarity). They are essential to limit the length of ubiquitin chains (By similarity).By similarity

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiKOG2935. Eukaryota.
ENOG4111G4B. LUCA.
GeneTreeiENSGT00390000001830.
HOGENOMiHOG000006034.
HOVERGENiHBG025648.
InParanoidiO35815.
KOiK11863.
OMAiMLQAAMN.
OrthoDBiEOG091G0OC6.
PhylomeDBiO35815.
TreeFamiTF314228.

Family and domain databases

Gene3Di1.10.287.10. 1 hit.
InterProiView protein in InterPro
IPR033865. Ataxin-3.
IPR006155. Josephin.
IPR009068. S15_NS1_RNA-bd.
IPR003903. UIM_dom.
PANTHERiPTHR14159. PTHR14159. 1 hit.
PfamiView protein in Pfam
PF02099. Josephin. 1 hit.
PF02809. UIM. 2 hits.
SMARTiView protein in SMART
SM01246. Josephin. 1 hit.
SM00726. UIM. 3 hits.
PROSITEiView protein in PROSITE
PS50957. JOSEPHIN. 1 hit.
PS50330. UIM. 2 hits.

Sequencei

Sequence statusi: Complete.

O35815-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERLRMAE
60 70 80 90 100
GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ
110 120 130 140 150
RLRIDPINER SFICNYKEHW FTVRKLGKQW FNLNSLLTGP ELISDTYLAL
160 170 180 190 200
FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IKVQQMHRPK LIGEELAHLK
210 220 230 240 250
EQSALKADLE RVLEAADGPG MFDDDEDDLQ RALAMSRQEI DMEDEEADLR
260 270 280 290 300
RAIQLSMQGS SRGMCEDSPQ TSSTDLSSEE LRKRREAYFE KQQHQQQEAD
310 320 330 340 350
RPGYLSYPCE RPTTSSGGLR SNQAGNAMSE EDVLRATVTV SLETAKDSLK

AERKK
Length:355
Mass (Da):40,446
Last modified:January 1, 1998 - v1
Checksum:i87B6A6EE54FF29F5
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Y12319 mRNA. Translation: CAA72986.1.
RefSeqiNP_067734.1. NM_021702.1.
UniGeneiRn.42932.

Genome annotation databases

EnsembliENSRNOT00000007505; ENSRNOP00000007505; ENSRNOG00000005470.
GeneIDi60331.
KEGGirno:60331.
UCSCiRGD:621567. rat.

Similar proteinsi

Entry informationi

Entry nameiATX3_RAT
AccessioniPrimary (citable) accession number: O35815
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 28, 2003
Last sequence update: January 1, 1998
Last modified: November 22, 2017
This is version 129 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome