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Tbio
ATXN3
Ataxin-3

Protein Summary
Description
Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:12297501, PubMed:17696782, PubMed:23625928, PubMed:28445460, PubMed:16118278). Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (PubMed:17696782). Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension (By similarity). Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription (PubMed:12297501). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed:28445460). Machado-Joseph disease, also known as spinocerebel ...more
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000340660
  • ENSP00000339110
  • ENSG00000066427
  • ENST00000502250
  • ENSP00000425322
  • ENST00000503767
  • ENSP00000426697
  • ENST00000532032
  • ENSP00000437157
  • ENST00000558190
  • ENSP00000478320
  • ENST00000644486
  • ENSP00000496695

Symbol
  • ATX3
  • MJD
  • MJD1
  • SCA3
  • AT3
  • JOS
  • MJD
  • ATX3
  • MJD1
  • SCA3
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
co-expressed gene
1
microRNA
0.84
biological process
0.8
virus perturbation
0.79
transcription factor binding site profile
0.78


Related Tools
Target Illumination GWAS Analytics (TIGA)
TIGA scores and ranks GWAS discovered associations according to the quantity and quality of the evidence supporting the association.
GENEVA
GENEVA (GENe Expression Variance Analysis) allows you to identify RNA-sequencing datasets from the Gene Expression Omnibus (GEO) that contain conditions modulating a gene or a gene signature.
GlyGen
GlyGen is a data integration and dissemination project for carbohydrate and glycoconjugate related data.
ARCHS4
ARCHS4 provides access to gene-function predictions based on RNA-seq co-expression, and gene expression levels across cell and tissues.
IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 535.05   (req: < 5)
Gene RIFs: 153   (req: <= 3)
Antibodies: 302   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 535.05   (req: >= 5)
Gene RIFs: 153   (req: > 3)
Antibodies: 302   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 29
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0
GWAS Traits (2)
GWAS Trait
EFO ID
Study Count
SNP Count
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
forced expiratory volume
1
1
1
41.8
vital capacity
1
1
1
21.2
GWAS Trait
EFO ID
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
forced expiratory volume
1
41.8
vital capacity
1
21.2
Orthologs (10)
1 – 5 of 10
Species
Name
Source ID
Gene ID
OMA
EggNOG
Inparanoid
Chimp
ataxin 3
VGNC:12976
738729
Macaque
ataxin 3
720515
Mouse
MGI:1099442
110616
Rat
RGD:621567
60331
Dog
ataxin 3
480229
Species
Name
OMA
EggNOG
Inparanoid
Chimp
ataxin 3
Macaque
ataxin 3
Mouse
Rat
Dog
ataxin 3
Protein Structure (7 Structures, 1 AlphaFold Model)
RepresentationColor Scheme

Click on a row in the table to change the structure displayed.
More information can be found at RCSB PDB

AF-P54252-F1-model_v1

AlphaFold Structures Developed by DeepMind and EMBL-EBI

1 – 5 of 7
PDB Structure Id
Ligand
Method
Resolution (Å)
Residues
Fraction of Total Protein
Pub Year
Title
PDB Structure Id
Fraction of Total Protein
Resolution
Pub Year
Pathways (10)
Deubiquitination (R-HSA-5688426)

Click on a row in the table to change the structure displayed.

Items per page:
1 – 5 of 9
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Deubiquitination
Reactome
FOXO-mediated transcription
Reactome
FOXO-mediated transcription of oxidative stress, metabolic and neuronal genes
Reactome
Gene expression (Transcription)
Reactome
Generic Transcription Pathway
Name
Explore in Pharos
Explore in Source
Deubiquitination
FOXO-mediated transcription
FOXO-mediated transcription of oxidative stress, metabolic and neuronal genes
Gene expression (Transcription)
Generic Transcription Pathway
Gene Ontology Terms (39)
Items per page:
10
1 – 9 of 9
GO Term
Evidence
Assigned by
Inferred from Direct Assay (IDA)
ParkinsonsUK-UCL
Inferred from Direct Assay (IDA)
ParkinsonsUK-UCL
Inferred from Physical Interaction (IPI)
ParkinsonsUK-UCL
Inferred from Physical Interaction (IPI)
IntAct
Inferred from Physical Interaction (IPI)
ParkinsonsUK-UCL
Inferred from Sequence or structural Similarity (ISS)
UniProtKB
Inferred from Sequence or structural Similarity (ISS)
UniProtKB
Inferred from Electronic Annotation (IEA)
Ensembl
Inferred from Electronic Annotation (IEA)
Ensembl
Protein-Protein Interactions (157)
1 – 10 of 157
TOMM20L
Tdark
Novelty: 4.50556416
p_int: 0.999999999
p_ni: 1e-9
Score: 0.485
Data Source: BioPlex,STRINGDB
IGF2
Tbio
Novelty: 0.00021277
p_int: 0.999996585
p_ni: 0.000002867
p_wrong: 5.48e-7
Data Source: BioPlex
TMEM31
Tdark
Novelty: 0.65381391
p_int: 0.999992214
p_ni: 0.000005918
p_wrong: 0.000001868
Data Source: BioPlex
DND1
Tbio
Novelty: 0.0287312
p_int: 0.999412306
p_ni: 0.000028795
p_wrong: 0.000558899
Score: 0.269
Data Source: BioPlex,STRINGDB
AEBP2
Tbio
Novelty: 0.05974483
p_int: 0.999131187
p_ni: 0.000045903
p_wrong: 0.00082291
Score: 0.308
Data Source: BioPlex,STRINGDB
UBB
Tbio
Novelty: 0.00661254
Score: 0.997
Data Source: STRINGDB
UBC
Tbio
Novelty: 0.00204643
Score: 0.997
Data Source: STRINGDB
UBA52
Tbio
Novelty: 0.01637744
Score: 0.995
Data Source: STRINGDB
VCP
Tchem
Family: Enzyme
Novelty: 0.00103317
Score: 0.994
Data Source: Reactome,STRINGDB
RAD23A
Tbio
Novelty: 0.01603801
Score: 0.989
Data Source: Reactome,STRINGDB
Publication Statistics
PubMed Score  535.05

PubMed score by year
PubTator Score  854.7

PubTator score by year
Amino Acid Sequence
Residue Counts
Protein Sequence
ProtVista Viewer
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