Atxn3 Gene Summary [Rat]

Enables RNA polymerase II transcription regulatory region sequence-specific DNA binding activity; histone deacetylase binding activity; and transcription corepressor binding activity. Contributes to histone deacetylase activity. Involved in protein modification process. Predicted to be located in several cellular components, including endoplasmic reticulum membrane; mitochondrion; and nucleus. Predicted to be active in lysosomal membrane and nucleus. Human ortholog(s) of this gene implicated in Machado-Joseph disease and late onset Parkinson's disease. Orthologous to human ATXN3 (ataxin 3). [provided by Alliance of Genome Resources, Apr 2025]

Details

Type
Protein Coding
Official Symbol
Atxn3
Official Name
ataxin 3 [Source:RGD Symbol;Acc:621567]
Ensembl ID
ENSRNOG00000005470
Bio databases IDs NCBI: 60331 Ensembl: ENSRNOG00000005470
Aliases ataxin 3
Synonyms 2210008M02Rik, AI463012, AT3, ATAXIN 3, ATX3, JOS, MJD, MJD1, Rsca3, SCA3
Species
Rat, Rattus norvegicus
OrthologiesHumanMouse

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in rat Atxn3 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • ubiquitin protein ligase binding
  • Ubiquitin interaction motif
  • nucleic acid binding
  • peptidase
  • Unstructured region C-term to UIM in Ataxin3
  • transcription factor binding
  • histone deacetylase binding
  • protein binding
  • sequence-specific DNA binding
  • identical protein binding
  • Josephin
  • ATPase binding
  • double-stranded DNA binding
  • ubiquitin binding domain
  • polyglutamine repeat
  • ubiquitin-specific protease
  • histone deacetylase
  • glutamine repeat domain
  • active site
  • ubiquitin-like-specific protease

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
  • neoplasia
  • chronic obstructive pulmonary disease
  • neurological disorder
  • lung adenocarcinoma
  • lung adenocarcinoma formation
  • ataxia
  • spinocerebellar ataxia type 3
  • melanoma cancer
  • coronary artery disease
  • late-onset Parkinson disease
regulated by
  • Y 27632
  • calpain
  • sodium dodecyl sulfate
  • Ca2+
  • KPNB1
  • caspase
  • L-glutamic acid
  • STUB1
  • simvastatin
  • beta-estradiol
regulates
role in cell
  • phosphorylation in
  • expression in
  • quantity
  • migration
  • apoptosis
  • production in
  • cell death
  • activation in
  • degradation in
  • accumulation in

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Nucleus
  • nuclear fraction
  • Cytoplasm
  • Plasma Membrane
  • cytosol
  • endoplasmic reticulum membrane
  • mitochondrial membrane
  • mitochondrial matrix
  • lysosome membrane
  • nucleoplasm
  • nucleoli

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the rat Atxn3 gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • protein deubiquitination
  • positive regulation of protein ubiquitination involved in ubiquitin-dependent protein catabolic process
  • monoubiquitinated protein deubiquitination
  • cellular response to amino acid starvation
  • nervous system development
  • intermediate filament cytoskeleton organization
  • nucleotide-excision repair
  • synaptic transmission
  • microtubule cytoskeleton organization
  • ubiquitin-dependent protein catabolic process
  • proteasomal ubiquitin-dependent protein catabolic process
  • cellular response to heat
  • misfolded or incompletely synthesized protein catabolic process
  • regulation of cell-substrate adhesion
  • actin cytoskeleton organization
  • protein K48-linked deubiquitination
  • protein K63-linked deubiquitination
  • cellular response to misfolded protein

Cellular Component

Where in the cell the gene product is active
  • nuclear inclusion body
  • synapse
  • nucleolus
  • plasma membrane
  • lysosomal membrane
  • nucleoplasm
  • endoplasmic reticulum membrane
  • nucleus
  • mitochondrial membrane
  • mitochondrial matrix
  • cytoplasm
  • cytosol
  • nuclear matrix

Molecular Function

What the gene product does at the molecular level
  • ubiquitin-specific protease activity
  • protein binding
  • ubiquitin protein ligase binding
  • ATPase binding

Gene-Specific Assays for Results You Can Trust

Streamline your workflow with assays designed for this gene. Our targeted dPCR and qPCR assays help you generate meaningful data – efficiently and accurately.