protein

ATAXIN3

Entity Detail — Knowledge Graph Node

Understanding Entity Pages

This page aggregates everything SciDEX knows about ATAXIN3: its mechanistic relationships (Knowledge Graph edges), hypotheses targeting it, analyses mentioning it, and supporting scientific papers. The interactive graph below shows its immediate neighbors. All content is AI-synthesized from peer-reviewed literature.

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🔬 Protein Info

Gene Symbol	ATAXIN3
Protein Family	Josephin family (deubiquitinating enzymes)
Protein Type	Enzyme
Function	is a deubiquitinating enzyme encoded by the ATXN3 gene.
Molecular Weight	42 kDa
UniProt ID	P54252
GeneCards	ATAXIN3
Human Protein Atlas	ATAXIN3
Protein quality control	Ataxin-3 removes polyubiquitin chains from misfolded proteins, facilitating their refolding or proteasomal degradation[@todi2022]
Transcriptional regulation	Ataxin-3 interacts with transcription factors including histone deacetylases and the androgen receptor, modulating gene expression[@evert2021]
Autophagy regulation	Ataxin-3 regulates autophagic flux by deubiquitinating autophagy receptors and maintaining protein aggregate clearance[@rui2022]
DNA repair	Ataxin-3 participates in DNA damage response pathways, including homologous recombination and base excision repair[@gao2023]
Mitochondrial quality control	Ataxin-3 helps maintain mitochondrial integrity and function under cellular stress[@liu2022]
Toxic gain-of-function	Expanded polyQ tract confers novel toxic properties, including altered protein interactions and impaired autophagy[@costa2023a]
Transcriptional dysregulation	Mutant ataxin-3 disrupts normal transcriptional programs through aberrant interactions with transcription factors and histone modifiers[@mcloughlin2022]
Associated Diseases	neurodegeneration
Databases	GeneCards NCBI Gene HPA STRING

🔮 Predicted Structure: ATAXIN3 — AlphaFold P54252 Click to expand

AI-predicted structure from AlphaFold | Powered by Mol*

Wiki Pages (1)

Knowledge base pages for this entity

Canonical Page

Ataxin-3 (Josephin)

protein · 3275 words

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Pathway Diagram

graph TD
    ATAXIN3["ATAXIN3
Deubiquitinating enzyme"]
    PolyQ["Polyglutamine expansion
CAG repeat mutations"]
    Aggregates["Protein aggregates
Nuclear inclusions"]
    UPS["Ubiquitin-proteasome
system dysfunction"]
    ER_stress["Endoplasmic reticulum
stress"]
    Mitochondria["Mitochondrial
dysfunction"]
    Autophagy["Autophagy
impairment"]
    Transcription["Transcriptional
dysregulation"]
    Apoptosis["Neuronal
apoptosis"]
    SCA3["Spinocerebellar ataxia
type 3 (SCA3)"]
    MJD["Machado-Joseph
disease"]
    Neurodegeneration["Neurodegeneration
Cerebellar atrophy"]
    HSPs["Heat shock proteins
Chaperone response"]
    p53["p53 tumor
suppressor"]

    PolyQ -->|"causes"| ATAXIN3
    ATAXIN3 -->|"forms"| Aggregates
    ATAXIN3 -->|"disrupts"| UPS
    UPS -->|"triggers"| ER_stress
    Aggregates -->|"activates"| ER_stress
    ER_stress -->|"impairs"| Mitochondria
    ATAXIN3 -->|"inhibits"| Autophagy
    Aggregates -->|"disrupts"| Transcription
    Transcription -->|"activates"| p53
    p53 -->|"triggers"| Apoptosis
    Mitochondria -->|"promotes"| Apoptosis
    ATAXIN3 -->|"causes"| SCA3
    ATAXIN3 -->|"causes"| MJD
    SCA3 -->|"leads to"| Neurodegeneration
    MJD -->|"leads to"| Neurodegeneration
    Apoptosis -->|"contributes to"| Neurodegeneration
    HSPs -->|"protects against"| Aggregates

    classDef central fill:#1a3a4a,stroke:#4fc3f7,stroke-width:3px,color:#fff
    classDef protective fill:#1a3a2a,stroke:#81c784,stroke-width:2px,color:#fff
    classDef pathological fill:#3a1a1a,stroke:#ef5350,stroke-width:2px,color:#fff
    classDef regulatory fill:#2a1a3a,stroke:#ce93d8,stroke-width:2px,color:#fff
    classDef disease fill:#3a2a1a,stroke:#ffa726,stroke-width:2px,color:#fff

    class ATAXIN3 central
    class HSPs protective
    class PolyQ,Aggregates,UPS,ER_stress,Mitochondria,Autophagy,Apoptosis,Neurodegeneration pathological
    class p53,Transcription regulatory
    class SCA3,MJD disease

Outgoing (0)

Target	Relation	Type	Str
No outgoing edges

Incoming (0)

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No incoming edges

Targeting Hypotheses (0)

Hypotheses where this entity is a therapeutic target

Hypothesis	Score	Disease	Analysis
No targeting hypotheses

Mentioning Analyses (0)

Scientific analyses that reference this entity

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Experiments (0)

Experimental studies targeting or related to this entity

Experiment	Type	Disease	Score	Feasibility	Model	Status	Est. Cost
No experiments found

Related Papers (0)

Scientific publications cited in analyses involving this entity

Title & PMID	Authors	Journal	Year	Citations
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Debates (0)

Multi-agent debates referencing this entity

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Related Research

Hypotheses and analyses mentioning ATAXIN3 in their description or question text

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