# ATRX

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

## TL;DR

ATRX (Chromatin remodeler ATRX) is a protein that switches other genes on and off. The public catalogues list it as a drug target, an oncogene driver, a tumour suppressor, a biomarker and a DNA repair gene, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Sarcomas, Neuroendocrine tumours, Adrenocortical carcinoma and 5 more.

## Summary

Involved in transcriptional regulation and chromatin remodeling. Facilitates DNA replication in multiple cellular environments and is required for efficient replication of a subset of genomic loci. Binds to DNA tandem repeat sequences in both telomeres and euchromatin and in vitro binds DNA quadruplex structures.

CIViC holds 11 clinical evidence items and 0 assertions across 6 variants, naming Temozolomide, Olaparib, Talazoparib and Cisplatin and others. Open Targets scores its association with cancer at 0.82 (direct and indirect evidence; datatypes literature 0.98, affected pathway 0.61, genetic association 0.72, somatic mutation 0.98). IntOGen calls it a driver in 27 cohorts (2 activating, 25 loss-of-function), covering Adrenocortical Carcinoma, Chronic Lymphocytic Leukaemia/Small Lymphocytic Lymphoma, Glioblastoma, Glioblastoma Multiforme, High-Grade Glioma, NOS, Low-Grade Glioma, NOS and others.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: ATRX chromatin remodeler; Chromatin remodeler ATRX; XH2; RAD54; MRX52
- Tags: cancer-genes-wave
- Symbol: ATRX
- Class: transcription
- Biology: Involved in transcriptional regulation and chromatin remodeling. Facilitates DNA replication in multiple cellular environments and is required for efficient replication of a subset of genomic loci. Binds to DNA tandem repeat sequences in both telomeres and euchromatin and in vitro binds DNA quadruplex structures. May help stabilising G-rich regions into regular chromatin structures by remodeling G4 DNA and incorporating H3.3-containing nucleosomes. Catalytic component of the chromatin remodeling complex ATRX:DAXX which has ATP-dependent DNA translocase activity and catalyses the replication-independent deposition of histone H3.3 in pericentric DNA repeats outside S-phase and telomeres, and the in vitro remodeling of H3.3-containing nucleosomes. Its heterochromatin targeting is proposed to involve a combinatorial readout of histone H3 modifications (specifically methylation states of H3K9 and H3K4) and association with CBX5. Location: Nucleus; Chromosome, telomere; Nucleus, PML body (UniProt). Locus Xq21.1 (HGNC).
- Where found: Sarcomas: Open Targets association 0.70 with sarcoma (MONDO_0005089); IntOGen driver in 5 cohorts (LMS, SARCNOS, SOFT_TISSUE); Neuroendocrine tumours: Open Targets association 0.63 with neuroendocrine neoplasm (MONDO_0019496); IntOGen driver in 1 cohort (PANET); Adrenocortical carcinoma: IntOGen driver in 3 cohorts (ACC); Renal cell carcinoma: Open Targets association 0.60 with renal cell carcinoma (MONDO_0005086); Ovarian cancer: IntOGen driver in 1 cohort (OVT); Breast cancer: Open Targets association 0.56 with breast cancer (MONDO_0007254)

## 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: CIViC lists 10 therapies; IntOGen calls it an activating (Act) driver in 2 cohorts; IntOGen calls it a loss-of-function (LoF) driver in 25 cohorts; CIViC holds 11 clinical evidence items on its variants; UniProt keyword "DNA repair". Evidence tier "clinical-evidence" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.
- Diseases the sources name that have no OnCo cancer page yet, so they are not linked: Malignant Astrocytoma; High-Grade Glioma, NOS; Low-Grade Glioma, NOS.

## Sources

- HGNC HGNC:886: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:886
- UniProt P46100: https://www.uniprot.org/uniprotkb/P46100/entry
- NCBI Gene 546: https://www.ncbi.nlm.nih.gov/gene/546
- Ensembl ENSG00000085224: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000085224

## Connected records

- collections: [CIViC](https://onco.cc/collections/civic/), [IntOGen](https://onco.cc/collections/intogen/), [Open Targets Platform](https://onco.cc/collections/open-targets/)
- cancers: [Adrenocortical carcinoma](https://onco.cc/cancers/adrenocortical/), [Breast cancer (all types)](https://onco.cc/cancers/breast-cancer/), [Colorectal cancer](https://onco.cc/cancers/colorectal/), [Neuroendocrine tumours](https://onco.cc/cancers/neuroendocrine/), [Ovarian cancer](https://onco.cc/cancers/ovarian/), [Renal cell carcinoma](https://onco.cc/cancers/rcc/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/), [Skin cancer (all types)](https://onco.cc/cancers/skin-cancer/)
- pathways: [Telomere maintenance & replicative immortality](https://onco.cc/pathways/telomere-maintenance/)

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