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Protein

Ataxin-3 homolog

Gene

atx-3

Organism
Caenorhabditis elegans
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Acts as chain editing deubiquitinating enzyme that binds and cleaves 'Lys-48'-linked polyubiquitin chains, with a preference for chains containing four or more ubiquitin molecules thereby modulating protein degradation by the ubiquitin-proteasome pathway (PubMed:19545544, PubMed:17234717, PubMed:21317884). Probably by regulating the IGF-1-insulin-like pathway, regulates lifespan (PubMed:21317884). Regulates germline DNA double-strand-break repair and apoptosis in response to DNA damage by recruiting E4 ubiquitin-protein ligase ufd-2 to DNA repair foci (PubMed:27669035). Interacts with key regulators of transcription and represses transcription (By similarity). Acts as a histone-binding protein that regulates transcription (By similarity).By similarity4 Publications

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).2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei20NucleophilePROSITE-ProRule annotation1
Active sitei117Proton acceptorPROSITE-ProRule annotation1
Active sitei132PROSITE-ProRule annotation1

GO - Molecular functioni

  • thiol-dependent ubiquitin-specific protease activity Source: WormBase

GO - Biological processi

  • chemical synaptic transmission Source: WormBase
  • protein deubiquitination Source: InterPro
  • regulation of transcription, DNA-templated Source: UniProtKB-KW
  • transcription, DNA-templated Source: UniProtKB-KW

Keywordsi

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

Enzyme and pathway databases

ReactomeiR-CEL-5689877 Josephin domain DUBs

Protein family/group databases

MEROPSiC86.003

Names & Taxonomyi

Protein namesi
Recommended name:
Ataxin-3 homolog (EC:3.4.19.122 Publications)
Alternative name(s):
Machado-Joseph disease-like protein
Gene namesi
Name:atx-3
ORF Names:F28F8.6
OrganismiCaenorhabditis elegansImported
Taxonomic identifieri6239 [NCBI]
Taxonomic lineageiEukaryotaMetazoaEcdysozoaNematodaChromadoreaRhabditidaRhabditoideaRhabditidaePeloderinaeCaenorhabditis
Proteomesi
  • UP000001940 Componenti: Chromosome V

Organism-specific databases

WormBaseiF28F8.6 ; CE09760 ; WBGene00006446 ; atx-3

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

Cytoplasm, Nucleus

Pathology & Biotechi

Disruption phenotypei

No visible phenotype (PubMed:17234717). In response to ionizing radiation-mediated DNA damage, the number of germline apoptotic corpses is increased, ufd-2 recruitment to nucleoli foci is impaired, the number of ubiquitin foci is increased and rad-51 retention to DNA damage foci is reduced; the increased germline apoptosis is suppressed in a ufd-2 (tm1380) mutant background (PubMed:27669035). At the higher temperature of 25 degrees Celsius, 60 percent of animals are uncoordinated (PubMed:19545544). Shows resistance to heat stress characterized by an increase in survival and expression of heat shock proteins such as hsp-16.2 and hsp-4 (PubMed:21526185). Expression of genes involved in the ubiquitin-proteasome pathway, motility, cell structure and signal transduction is affected (PubMed:17234717). In a cdc-48.1 (tm544) mutant background, causes a 50 percent increase in longevity, a delay in age-related muscle degeneration and resistance to oxidative and heat stresses (PubMed:21317884). In addition, induces dauer formation in 10 percent of animals and increases gene transcription of several genes including sod-3 and hsp-16.2 (PubMed:21317884). The overall levels of polyubiquitinated proteins is not affected (PubMed:21317884). RNAi-mediated knockdown of daf-16 abolishes the increase in lifespan (PubMed:21317884).5 Publications

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi20C → A: Loss of catalytic activity. In a cdc-48.1 (tm544) mutant background, causes an increase in longevitiy and resistance to oxidative stress. 2 Publications1
Mutagenesisi232S → A: Loss of catalytic activity. 1 Publication1
Mutagenesisi260S → A: Loss of catalytic activity. 1 Publication1
Mutagenesisi296 – 299RRDR → HNHH: Loss of interaction with cdc-48.1 and cdc-48.2. 1 Publication4

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000538351 – 317Ataxin-3 homologAdd BLAST317

Proteomic databases

EPDiO17850
PaxDbiO17850
PeptideAtlasiO17850

PTM databases

iPTMnetiO17850

Expressioni

Tissue specificityi

Expressed in germline (at protein level) (PubMed:27669035). Expressed in spermatheca, pharynx, dorsal and ventral cords, some head neurons, hypodermis, body wall muscles and coelomocytes (PubMed:17234717).2 Publications

Developmental stagei

Expression begins during late embryogenesis and continues in larvae and adults.1 Publication

Gene expression databases

BgeeiWBGene00006446

Interactioni

Subunit structurei

Forms a complex composed of deubiquitinating enzyme atx-3, adapter ubxn-5 and cdc-48.1 (PubMed:19545544). Forms a complex composed of deubiquitinating enzyme atx-3, E4 ubiquitin-protein ligase ufd-2 and cdc-48.1 (PubMed:21317884). Interacts (via RRDR motif) with cdc-48.1 (via N-terminus) and cdc-48.2 (via N-terminus); the interaction with cdc-48.1 is not required for atx-3 enzymatic activity (PubMed:19545544, PubMed:21317884). Interacts (via C-terminus) with ubxn-5 (PubMed:19545544). May interact with ned-8 (PubMed:17935801).3 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
cdc-48.2P548123EBI-320650,EBI-320265

Protein-protein interaction databases

BioGridi45043, 4 interactors
DIPiDIP-27501N
IntActiO17850, 3 interactors
STRINGi6239.F28F8.6.1

Structurei

3D structure databases

ProteinModelPortaliO17850
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini7 – 178JosephinPROSITE-ProRule annotationAdd BLAST172
Domaini219 – 239UIM 1CuratedAdd BLAST21
Domaini247 – 264UIM 2CuratedAdd BLAST18

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni296 – 299Interaction with cdc-48.1 and cdc-48.21 Publication4

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiKOG2935 Eukaryota
ENOG4111G4B LUCA
GeneTreeiENSGT00390000001830
HOGENOMiHOG000006034
InParanoidiO17850
KOiK11863
OMAiHIFIVEG
OrthoDBiEOG091G0OC6
PhylomeDBiO17850

Family and domain databases

InterProiView protein in InterPro
IPR033865 Ataxin-3
IPR006155 Josephin
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
PROSITEiView protein in PROSITE
PS50957 JOSEPHIN, 1 hit

Sequencei

Sequence statusi: Complete.

O17850-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSKDDPINSI FFEHQEAALC AQHALNMLLQ DALYKWQDLR DLAIQMDKME
60 70 80 90 100
QQILGNANPT PGRSENMNES GYFSIQVLEK ALETFSLKLT NIENPAMVDY
110 120 130 140 150
KNNPLTARAY ICNLREHWFV LRKFGNQWFE LNSVNRGPKL LSDTYVSMFL
160 170 180 190 200
HQVSSEGYSI FVVQGVLPRS DADDLISLCP VVPPKVTPKK EQKLEKVMTK
210 220 230 240 250
FFNTVGKRLG GGSGAPPDSQ EEKDLAIAFA MSMETKDGSE VSRSSAEIDE
260 270 280 290 300
ENLRKAIELS QAPGPSEPAE IPLLTRSRSS TPPGASEPFS NAEQQRRDRQ
310
KFLERFEKKK EERNDEK
Length:317
Mass (Da):35,864
Last modified:January 1, 1998 - v1
Checksum:i31798FD62667FB19
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z81071 Genomic DNA Translation: CAB03016.1
PIRiT21511
RefSeqiNP_506873.1, NM_074472.7
UniGeneiCel.6510

Genome annotation databases

EnsemblMetazoaiF28F8.6.1; F28F8.6.1; WBGene00006446
F28F8.6.2; F28F8.6.2; WBGene00006446
F28F8.6.3; F28F8.6.3; WBGene00006446
GeneIDi180049
KEGGicel:CELE_F28F8.6
UCSCiF28F8.6.1 c. elegans

Similar proteinsi

Entry informationi

Entry nameiATX3_CAEEL
AccessioniPrimary (citable) accession number: O17850
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 28, 2003
Last sequence update: January 1, 1998
Last modified: May 23, 2018
This is version 126 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programCaenorhabditis annotation project

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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