Q9W689 (ATX3_CHICK) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Ataxin-3
EC=3.4.19.12

Alternative name(s):
Machado-Joseph disease protein 1 homolog

Gene names

Name:	ATXN3
Synonyms:	MDJ1, MJD

Organism

Gallus gallus (Chicken) [Reference proteome]

Taxonomic identifier

9031 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Sauropsida › Sauria › Archosauria › Dinosauria › Saurischia › Theropoda › Coelurosauria › Aves › Neognathae › Galliformes › Phasianidae › Phasianinae › Gallus

Protein attributes

Sequence length	363 AA.
Sequence status	Complete.
Protein existence	Evidence at transcript level

General annotation (Comments)

Function	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 activity	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).
Subcellular location	Nucleus By similarity.
Tissue specificity	Widely expressed.
Sequence similarities	Contains 1 Josephin domain. Contains 3 UIM (ubiquitin-interacting motif) repeats.

Ontologies

Keywords
Biological process	Transcription Transcription regulation Ubl conjugation pathway
Cellular component	Nucleus
Domain	Repeat
Molecular function	Hydrolase Protease Thiol protease
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	cellular response to heat Inferred from electronic annotation. Source: Compara cellular response to misfolded protein Inferred from sequence or structural similarity. Source: UniProtKB exploration behavior Inferred from electronic annotation. Source: Compara misfolded or incompletely synthesized protein catabolic process Inferred from sequence or structural similarity. Source: UniProtKB monoubiquitinated protein deubiquitination Inferred from sequence or structural similarity. Source: UniProtKB proteasomal ubiquitin-dependent protein catabolic process Inferred from sequence or structural similarity. Source: UniProtKB regulation of cell-substrate adhesion Inferred from electronic annotation. Source: Compara regulation of transcription, DNA-dependent Inferred from electronic annotation. Source: UniProtKB-KW transcription, DNA-dependent Inferred from electronic annotation. Source: UniProtKB-KW
Cellular_component	mitochondrial matrix Inferred from electronic annotation. Source: Compara mitochondrial membrane Inferred from electronic annotation. Source: Compara nuclear inclusion body Inferred from electronic annotation. Source: Compara
Molecular_function	omega peptidase activity Inferred from electronic annotation. Source: InterPro ubiquitin protein ligase binding Inferred from sequence or structural similarity. Source: UniProtKB ubiquitin thiolesterase activity Inferred from sequence or structural similarity. Source: UniProtKB ubiquitin-specific protease activity Inferred from sequence or structural similarity. Source: UniProtKB
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 363	363	Ataxin-3		PRO_0000053834
Regions
	Domain	1 – 180	180	Josephin
	Repeat	227 – 246	20	UIM 1
	Repeat	247 – 266	20	UIM 2
	Repeat	337 – 356	20	UIM 3
	Compositional bias	294 – 297	4	Poly-Arg
	Compositional bias	304 – 311	8	Poly-Gln
Sites
	Active site	14	1	Nucleophile By similarity
	Active site	119	1	Proton acceptor By similarity
	Active site	134	1	By similarity

Sequences

	Sequence		Length	Mass (Da)
	Q9W689 [UniParc]. Last modified November 1, 1999. Version 1. Checksum: 15C81537096DE8C4	FASTA	363	41,608
	10 20 30 40 50 60 MESIFHERQE GSLCAQHCLN NLLQGEYFSP VELSSIAQQL DEEERMRMAE GGVSSEEYRT 70 80 90 100 110 120 FLQQPSVNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ RLGIDPINEK SFICNYKEHW 130 140 150 160 170 180 FTVRKLGKQW FNLNSLLMGP ELISDTYLAL FLAQLQQEGY SIFVVKGDLP DCEADQLLQM 190 200 210 220 230 240 IRVQQVQRPK LIGEETAQSR DQRLPRSDVD QAIEVSHPFD GTGMLDEDEE NFQRALALSR 250 260 270 280 290 300 QEIDMEDEEA DLRRAIQLSM QGSRQSEFSN SLPQNASQPP HTSQTDSLSS EDLRRRRQAY 310 320 330 340 350 360 FEKQQQQLQQ QDLTLNLHDK PTINSSTLEA DPGGDMSEED MLQAAMNMSL ESARNHLSTE EKK

References

[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.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AF085247 mRNA. Translation: AAD21923.1.
IPI	IPI00599336.
RefSeq	NP_989688.1. NM_204357.1.
UniGene	Gga.44490.
3D structure databases
ProteinModelPortal	Q9W689.
SMR	Q9W689. Positions 1-185.
ModBase	Search...
Protein family/group databases
MEROPS	C86.001.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENSGALT00000017509; ENSGALP00000017488; ENSGALG00000010766.
GeneID	378424.
KEGG	gga:378424.
Organism-specific databases
CTD	4287.
Phylogenomic databases
eggNOG	NOG327234.
GeneTree	ENSGT00390000001830.
HOGENOM	HOG000006034.
HOVERGEN	HBG025648.
InParanoid	Q9W689.
KO	K11863.
OMA	LQAAMNM.
Gene expression databases
ArrayExpress	Q9W689.
Family and domain databases
InterPro	IPR006155. Josephin. IPR003903. Ubiquitin-int_motif. [Graphical view]
Pfam	PF02099. Josephin. 1 hit. PF02809. UIM. 3 hits. [Graphical view]
PRINTS	PR01233. JOSEPHIN.
SMART	SM00726. UIM. 3 hits. [Graphical view]
PROSITE	PS50957. JOSEPHIN. 1 hit. PS50330. UIM. 3 hits. [Graphical view]
ProtoNet	Search...
Other
NextBio	20813765.

Entry information

Entry name

ATX3_CHICK

Accession

Primary (citable) accession number: Q9W689

Entry history

Integrated into UniProtKB/Swiss-Prot:	February 28, 2003
Last sequence update:	November 1, 1999
Last modified:	April 3, 2013
This is version 78 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program