# SIX1

Source: https://onco.cc/targets/six1/  
OnCo record `six1` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

SIX1 (Homeobox protein SIX1) is a gene that drives cell growth when it is altered. The public catalogues list it as an oncogene driver, and it is called a cancer driver by mutation analysis of patient cohorts. Tied to Colorectal cancer and Wilms tumour.

## Summary

Transcription factor that is involved in the regulation of cell proliferation, apoptosis and embryonic development. Plays an important role in the development of several organs, including kidney, muscle and inner ear. Depending on context, functions as a transcriptional repressor or activator.

Open Targets scores its association with cancer at 0.73 (direct and indirect evidence; datatypes literature 0.99, animal model 0.49, genetic association 0.51, somatic mutation 0.89). IntOGen calls it a driver in 1 cohort (1 activating, 0 loss-of-function), covering Wilms' Tumour.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: SIX homeobox 1; Homeobox protein SIX1; DFNA23
- Tags: cancer-genes-wave
- Symbol: SIX1
- Class: oncogene
- Biology: Transcription factor that is involved in the regulation of cell proliferation, apoptosis and embryonic development. Plays an important role in the development of several organs, including kidney, muscle and inner ear. Depending on context, functions as a transcriptional repressor or activator. Lacks an activation domain, and requires interaction with EYA family members for transcription activation. Mediates nuclear translocation of EYA1 and EYA2. Binds the 5'-TCA[AG][AG]TTNC-3' motif present in the MEF3 element in the MYOG promoter and CIDEA enhancer. Location: Nucleus; Cytoplasm (UniProt). Locus 14q23.1 (HGNC).
- Where found: Colorectal cancer: Open Targets association 0.52 with colorectal cancer (MONDO_0005575); Wilms tumour: IntOGen driver in 1 cohort (WT)

## 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: IntOGen calls it an activating (Act) driver in 1 cohort. Evidence tier "cohort-driver" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.

## Sources

- HGNC HGNC:10887: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:10887
- UniProt Q15475: https://www.uniprot.org/uniprotkb/Q15475/entry
- NCBI Gene 6495: https://www.ncbi.nlm.nih.gov/gene/6495
- Ensembl ENSG00000126778: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000126778

## Connected records

- collections: [IntOGen](https://onco.cc/collections/intogen/), [Open Targets Platform](https://onco.cc/collections/open-targets/)
- cancers: [Colorectal cancer](https://onco.cc/cancers/colorectal/), [Wilms tumour (nephroblastoma)](https://onco.cc/cancers/wilms-tumor/)

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JSON: https://onco.cc/api/v1/entities/six1.json