ATXN3
ataxin 3 | ATX3, JOS, MJD, SCA3

Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jul 2016]

Member of: DE-2
Biological processes 62 terms
ATPase binding (GO:0051117)K48-linked deubiquitinase activity (GO:1990380)K63-linked deubiquitinase activity (GO:0061578)actin cytoskeleton organization (GO:0030036)cellular response to amino acid starvation (GO:0034198)cellular response to heat (GO:0034605)cellular response to misfolded protein (GO:0071218)cellular response to misfolded protein (GO:0071218)chemical synaptic transmission (GO:0007268)cysteine-type deubiquitinase activity (GO:0004843)cysteine-type deubiquitinase activity (GO:0004843)cysteine-type deubiquitinase activity (GO:0004843)cysteine-type deubiquitinase activity (GO:0004843)cysteine-type deubiquitinase activity (GO:0004843)cytoplasm (GO:0005737)cytoplasm (GO:0005737)cytosol (GO:0005829)cytosol (GO:0005829)endoplasmic reticulum membrane (GO:0005789)identical protein binding (GO:0042802)intermediate filament cytoskeleton organization (GO:0045104)lysosomal membrane (GO:0005765)lysosomal membrane (GO:0005765)microtubule cytoskeleton organization (GO:0000226)mitochondrial matrix (GO:0005759)mitochondrial matrix (GO:0005759)mitochondrial membrane (GO:0031966)mitochondrial membrane (GO:0031966)monoubiquitinated protein deubiquitination (GO:0035520)monoubiquitinated protein deubiquitination (GO:0035520)negative regulation of TORC1 signaling (GO:1904262)negative regulation of TORC1 signaling (GO:1904262)nervous system development (GO:0007399)nuclear inclusion body (GO:0042405)nuclear inclusion body (GO:0042405)nuclear matrix (GO:0016363)nucleoplasm (GO:0005654)nucleotide-excision repair (GO:0006289)nucleus (GO:0005634)nucleus (GO:0005634)nucleus (GO:0005634)positive regulation of ERAD pathway (GO:1904294)positive regulation of ERAD pathway (GO:1904294)positive regulation of ubiquitin-dependent protein catabolic process (GO:2000060)proteasome-mediated ubiquitin-dependent protein catabolic process (GO:0043161)proteasome-mediated ubiquitin-dependent protein catabolic process (GO:0043161)proteasome-mediated ubiquitin-dependent protein catabolic process (GO:0043161)protein K48-linked deubiquitination (GO:0071108)protein K63-linked deubiquitination (GO:0070536)protein binding (GO:0005515)protein deubiquitination (GO:0016579)protein deubiquitination (GO:0016579)protein localization to cytosolic proteasome complex (GO:1904327)protein quality control for misfolded or incompletely synthesized proteins (GO:0006515)protein quality control for misfolded or incompletely synthesized proteins (GO:0006515)protein quality control for misfolded or incompletely synthesized proteins (GO:0006515)regulation of cell-substrate adhesion (GO:0010810)synapse (GO:0045202)ubiquitin protein ligase binding (GO:0031625)ubiquitin protein ligase binding (GO:0031625)ubiquitin protein ligase binding (GO:0031625)ubiquitin-dependent protein catabolic process (GO:0006511)
Expression (TPM)
ATXN3 — as a Regulated Gene

TFs regulating ATXN3 0 TFs

Transcription factors with Perturb-seq knockdown data for ATXN3. The Binding column indicates whether any binding evidence exists for this TF–gene pair (ChIP-seq or motif footprint peaks). The Mean coef is the average Perturb-seq regression coefficient across active gRNAs (positive = ATXN3 upregulated upon KD; negative = downregulated). The Outlier column indicates whether this gene is in the top or bottom 5% of all TF knockdown effects.

Data: Effect:
TF Mean coef Binding Outlier TF→Gene link

Elements linked to ATXN3

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of ATXN3, linked by TSS proximity or chromatin conformation (Multiome / HiCAR). Each element overlaps at least one TF ChIP-seq binding site — the TFs column shows how many distinct TFs bind that element.

Accessibility Element Dist. to TSS Link type TFs
chr14:91,835,675–91,837,066 270.0 kb Distal (>10kb) Multiome 412
chr14:91,947,236–91,948,135 158.9 kb Distal (>10kb) Multiome 316
chr14:92,039,326–92,040,502 66.5 kb Distal (>10kb) Multiome 937
chr14:92,105,904–92,107,104 47 bp At TSS Multiome 794
chr14:92,108,368–92,108,782 1.8 kb Proximal (<10kb) 154
chr14:92,121,241–92,122,514 15.3 kb Distal (>10kb) Multiome 838
chr14:92,322,882–92,323,968 216.9 kb Distal (>10kb) Multiome 231

Genome Browser

Genomic view of the ATXN3 locus showing ATAC-seq accessibility and RNA-seq expression across the ESC → DE time course, together with TF binding peaks and element-to-TSS loop connections.

chr14:91,825,675 – 92,333,968
Proximal 1 kb Distal 10 kb Multiome HiCAR ATAC-seq RNA-seq