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Gene: Atxn3 (ataxin 3) Mus musculus
Symbol: Atxn3
Name: ataxin 3
Description: Exhibits thiol-dependent ubiquitin-specific protease activity and ubiquitin protein ligase binding activity. Predicted to contribute to histone deacetylase activity. Involved in several processes, including cellular response to misfolded protein; exploration behavior; and proteolysis. Localizes to the mitochondrial matrix; mitochondrial membrane; and nuclear inclusion body. Predicted to colocalize with the endoplasmic reticulum membrane. Human ortholog(s) of this gene implicated in Machado-Joseph disease. Is expressed in several structures, including alimentary system; brain; hemolymphoid system gland; musculature; and testis. Orthologous to several human genes including ATXN3 (ataxin 3); PARTICIPATES IN mitochondrial autophagy pathway; Endoplasmic Reticulum-associated degradation pathway; INTERACTS WITH 17alpha-ethynylestradiol; 2,3,7,8-tetrachlorodibenzodioxine; 4-hydroxyphenyl retinamide.
Type: protein-coding
RefSeq Status: VALIDATED
Also known as: 2210008M02Rik; AI463012; AI647473; ataxin-3; ATX3; expressed sequence AI463012; Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) homolog; machado-Joseph disease protein 1 homolog; Mjd; MJD1; Sca3
Homo sapiens (human) : ATXN3 (ataxin 3)  HGNC  Alliance
Rattus norvegicus (Norway rat) : Atxn3 (ataxin 3)  Alliance
Chinchilla lanigera (long-tailed chinchilla) : Atxn3 (ataxin 3)
Pan paniscus (bonobo/pygmy chimpanzee) : ATXN3 (ataxin 3)
Canis lupus familiaris (dog) : ATXN3 (ataxin 3)
Ictidomys tridecemlineatus (thirteen-lined ground squirrel) : Atxn3 (ataxin 3)
Sus scrofa (pig) : ATXN3 (ataxin 3)
Chlorocebus sabaeus (green monkey) : ATXN3 (ataxin 3)
Heterocephalus glaber (naked mole-rat) : Atxn3 (ataxin 3)
more info ...
Latest Assembly: GRCm38 - Mouse Genome Assembly GRCm38
Mouse AssemblyChrPosition (strand)SourceGenome Browsers
GRCm38 Ensembl12101,918,901 - 101,958,246 (-)Ensembl
GRCm3812101,918,901 - 101,959,121 (-)NCBIGRCm38GRCm38mm10GRCm38
MGSCv3712103,157,111 - 103,196,453 (-)NCBIGRCm37mm9NCBIm37
MGSCv3612102,320,604 - 102,359,232 (-)NCBImm8
Celera12103,135,861 - 103,175,388 (-)NCBICelera
Cytogenetic Map12 ENCBI
JBrowse: View Region in Genome Browser (JBrowse)

Disease Annotations
Gene-Chemical Interaction Annotations
Gene Ontology Annotations
Molecular Pathway Annotations
Phenotype Annotations
References - curated
References - uncurated


Comparative Map Data
Position Markers
QTLs in Region (GRCm38)
miRNA Target Status



Nucleotide Sequences
Protein Sequences

Additional Information

External Database Links
Nomenclature History
More on Atxn3
Alliance Gene
Ensembl Gene
JBrowse: mm9 mm10
MGI Report
NCBI Genome Data Viewer

RGD Object Information
RGD ID: 1332234
Created: 2005-02-01
Species: Mus musculus
Last Modified: 2020-02-04
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.