# PAX3

Source: https://onco.cc/targets/pax3/  
OnCo record `pax3` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

PAX3 (Paired box protein Pax-3) is a protein that switches other genes on and off. The public catalogues list it as a fusion partner, and the evidence so far is association rather than a proven role. Tied to Sarcomas and Rhabdomyosarcoma.

## Summary

Transcription factor that may regulate cell proliferation, migration and apoptosis. Involved in neural development and myogenesis. Transcriptional activator of MITF, acting synergistically with SOX10.

Open Targets scores its association with cancer at 0.79 (direct and indirect evidence; datatypes genetic literature 0.61, affected pathway 0.40, literature 0.96, genetic association 0.75, somatic mutation 0.98, animal model 0.57).

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: paired box 3; Paired box protein Pax-3; HUP2; PAX-3; WS1
- Tags: cancer-genes-wave
- Symbol: PAX3
- Class: transcription
- Biology: Transcription factor that may regulate cell proliferation, migration and apoptosis. Involved in neural development and myogenesis. Transcriptional activator of MITF, acting synergistically with SOX10. Location: Nucleus (UniProt). Locus 2q36.1 (HGNC).
- Where found: Sarcomas: Open Targets association 0.77 with sarcoma (MONDO_0005089); Rhabdomyosarcoma: Open Targets association 0.74 with rhabdomyosarcoma (MONDO_0005212)

## Notes

- Written by scripts/fetch-cancer-genes.ts from CIViC, Open Targets, IntOGen, HGNC and UniProt; the function text is UniProt's, condensed and in UK spelling. Roles: UniProt disease notes describe a translocation or gene fusion involving the gene. Evidence tier "association-only" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.

## Sources

- HGNC HGNC:8617: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:8617
- UniProt P23760: https://www.uniprot.org/uniprotkb/P23760/entry
- NCBI Gene 5077: https://www.ncbi.nlm.nih.gov/gene/5077
- Ensembl ENSG00000135903: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000135903

## Connected records

- collections: [Open Targets Platform](https://onco.cc/collections/open-targets/)
- cancers: [Rhabdomyosarcoma](https://onco.cc/cancers/rhabdomyosarcoma/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/)

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JSON: https://onco.cc/api/v1/entities/pax3.json