# GLI1

Source: https://onco.cc/targets/gli1/  
OnCo record `gli1` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

GLI1 (Transcription activator GLI1) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as a tumour suppressor, and it is called a cancer driver by mutation analysis of patient cohorts. Tied to Sarcomas and Leiomyosarcoma.

## Summary

Transcription activator that acts as a key effector of the smoothened signalling pathway, and which plays a role in craniofacial development and digital development, as well as development of the central nervous system and gastrointestinal tract. Binds to the DNA consensus sequence 5'-GACCACCCA-3'. Activated in response to smoothened signalling: in presence of hedgehog (DHH, IHH or SHH) and subsequent activation of smoothened, GLI1 translocates to the nucleus and promotes expression of target genes.

IntOGen calls it a driver in 1 cohort (0 activating, 1 loss-of-function), covering Leiomyosarcoma.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: GLI family zinc finger 1; Transcription activator GLI1
- Tags: cancer-genes-wave
- Symbol: GLI1
- Class: tumor-suppressor
- Biology: Transcription activator that acts as a key effector of the smoothened signalling pathway, and which plays a role in craniofacial development and digital development, as well as development of the central nervous system and gastrointestinal tract. Binds to the DNA consensus sequence 5'-GACCACCCA-3'. Activated in response to smoothened signalling: in presence of hedgehog (DHH, IHH or SHH) and subsequent activation of smoothened, GLI1 translocates to the nucleus and promotes expression of target genes. In contrast to GLI2 and GLI3, does not contain a repressor domain and only acts as a transcription activator. Acts as a transcriptional activator, but activates a different set of genes than isoform 1. Activates expression of CD24, unlike isoform 1. Location: Cytoplasm; Nucleus; Cell projection, cilium (UniProt). Locus 12q13.3 (HGNC).
- Where found: Sarcomas: IntOGen driver in 1 cohort (LMS); Leiomyosarcoma: IntOGen driver in 1 cohort (LMS)

## 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 a loss-of-function (LoF) 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:4317: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:4317
- UniProt P08151: https://www.uniprot.org/uniprotkb/P08151/entry
- NCBI Gene 2735: https://www.ncbi.nlm.nih.gov/gene/2735
- Ensembl ENSG00000111087: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000111087

## Connected records

- collections: [IntOGen](https://onco.cc/collections/intogen/)
- cancers: [Leiomyosarcoma](https://onco.cc/cancers/leiomyosarcoma/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/)
- pathways: [Basal cell carcinoma (KEGG map)](https://onco.cc/pathways/basal-cell-carcinoma-signalling/), [Hedgehog signalling](https://onco.cc/pathways/hedgehog/)

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JSON: https://onco.cc/api/v1/entities/gli1.json