Gene

ATRX

ATRX chromatin remodeler

Explore in graph →CI-GENE-00021460HGNC:886 ENSG00000085224 NCBI 546 CIViC Cancer geneApproved

Curated evidence

Clinical evidence (13)

CIViC items involving this gene, grouped by molecular profile and therapy, with native levels and directions. 50 items per page.

civicCuratedShowing 1–13 of 13 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.

Therapy	Cancer	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
ATRX Deletion1
Cisplatin + FluorouracilSubstitutes	Malignant NeoplasmALIAS	Predictive	D	Supports

Cohorts

Alteration frequency by cohort (200)

Frequency = cases affected / cases profiled within one cohort. Cohorts are never pooled.

cbioportalObserved200 cohort rows (showing 50, sorted by frequency) · frequency = affected / profiled, as published by each cohort; cohorts are not pooled. Hover a row badge for its release.

Cohort	Mapped cancer	Alteration	Affected (n)	Profiled (n)	Frequency (%)	Rank in cohort	Source
Low-Grade Gliomas (UCSF, Science 2014) lgg_ucsf_2014	Diffuse Glioma	Ssm	50	61

Literature

Linked publications (5)

25 per page, newest first.

pubmedPublishedShowing 1–5 of 5 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

Pediatric low-grade gliomas can be molecularly stratified for risk.
· Acta Neuropathol · 2018 · PMID 29948154 · validated civic_curation pubmed
ATRX loss refines the classification of anaplastic gliomas and identifies a subgroup of IDH mutant astrocytic tumors with better prognosis.
· Acta Neuropathol · 2013 · PMID 23904111 · validated civic_curation pubmed
Whole-exome sequencing identifies ATRX mutation as a key molecular determinant in lower-grade glioma.
· Oncotarget · 2012 · PMID 23104868 · validated civic_curation pubmed
ATRX loss promotes tumor growth and impairs nonhomologous end joining DNA repair in glioma.
· Sci Transl Med · 2016 · PMID 26936505 · validated civic_curation

ATRX Loss-of-function4
(prognostic)	Neuroblastoma	Prognostic	B	Does Not Support Poor Outcome	3	submitted	EID13290 In a Children's Oncology Group correlative study, 242 pretherapy tumours from patients enrolled on the phase III high-risk neuroblastoma trial ANBL0532 (NCT00567567) underwent ultra-deep targeted sequ… (full text at CIViC) PMID 40036726 · Berko et al., 2025 · Open in CIViC	civic
Adavosertib	GlioblastomaCURATED_BROADER	Predictive	D	Supports Sensitivity Response	2	submitted	EID10939 ATRX deficient glioblastoma model cell lines were created using CRISPR-based gene editing to knock-out ATRX in immortalized astrocytes. ATRX knock-out cells were found to be sensitive (IC50 0.012 uM) … (full text at CIViC) PMID 34118569 · Garbarino et al., 2021 · Open in CIViC	civic
Olaparib + TalazoparibSubstitutes	GlioblastomaCURATED_BROADER	Predictive	D	Supports Sensitivity Response	4	submitted	EID10940 ATRX deficient glioblastoma model cell lines were created using CRISPR-based gene editing to knock-out ATRX in immortalized astrocytes. ATRX knock-out cells were found to be more sensitive than wild-t… (full text at CIViC) PMID 34118569 · Garbarino et al., 2021 · Open in CIViC	civic
Pyridostatin	GlioblastomaCURATED_BROADER	Predictive	D	Supports Sensitivity Response	2	submitted	EID10938 ATRX deficient glioblastoma model cell lines were created using CRISPR-based gene editing to knock-out ATRX in immortalized astrocytes. The ATRX knock-out cells were found to be particularly sensitive… (full text at CIViC) PMID 34118569 · Garbarino et al., 2021 · Open in CIViC	civic

ATRX Loss-of-function4

(prognostic)

Neuroblastoma

Prognostic

Does Not Support Poor Outcome

submitted

EID13290

In a Children's Oncology Group correlative study, 242 pretherapy tumours from patients enrolled on the phase III high-risk neuroblastoma trial ANBL0532 (NCT00567567) underwent ultra-deep targeted sequ… (full text at CIViC)

PMID 40036726 · Berko et al., 2025 · Open in CIViC

civic

Adavosertib

GlioblastomaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID10939

ATRX deficient glioblastoma model cell lines were created using CRISPR-based gene editing to knock-out ATRX in immortalized astrocytes. ATRX knock-out cells were found to be sensitive (IC50 0.012 uM) … (full text at CIViC)

PMID 34118569 · Garbarino et al., 2021 · Open in CIViC

civic

Olaparib + TalazoparibSubstitutes

GlioblastomaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID10940

ATRX deficient glioblastoma model cell lines were created using CRISPR-based gene editing to knock-out ATRX in immortalized astrocytes. ATRX knock-out cells were found to be more sensitive than wild-t… (full text at CIViC)

PMID 34118569 · Garbarino et al., 2021 · Open in CIViC

civic

Pyridostatin

GlioblastomaCURATED_BROADER

Predictive

Supports Sensitivity Response

submitted

EID10938

ATRX deficient glioblastoma model cell lines were created using CRISPR-based gene editing to knock-out ATRX in immortalized astrocytes. The ATRX knock-out cells were found to be particularly sensitive… (full text at CIViC)

PMID 34118569 · Garbarino et al., 2021 · Open in CIViC

civic

ATRX Mutation4
(diagnostic)	Anaplastic AstrocytomaALIAS	Diagnostic	B	Supports Positive	5	submitted	EID8950 293 adult cases of low grade gliomas (LGGs) underwent a variety of analyses, including but not limited to exome sequencing (289 samples), copy number profiling (285), mRNA sequencing (277) and sequenc… (full text at CIViC) PMID 26061751 · 2015, N. Engl. J. Med. · Open in CIViC	civic
〃	Brain GliomaUNRESOLVED	Diagnostic	B	Supports Positive	3	rejected	EID8866 The study examined 4 cases of low grade glioma (LGG) with whole exome sequencing, followed by targeted sequencing of 28 LGGs. The study demonstrated that all IDH-mutant LGG with intact 1p/19q status h… (full text at CIViC) PMID 23104868 · Kannan et al., 2012 · Open in CIViC	civic
〃	Brain GliomaUNRESOLVED	Diagnostic	B	Supports Positive	3	rejected	EID8867 The study examined 4 cases of low grade glioma (LGG) with whole exome sequencing, followed by targeted sequencing of 28 LGGs. The study demonstrated that all IDH-mutant LGG with intact 1p/19q status h… (full text at CIViC) PMID 23104868 · Kannan et al., 2012 · Open in CIViC	civic
〃	Anaplastic AstrocytomaALIAS	Diagnostic	B	Supports Positive	3	accepted	EID8868 Whole exome sequencing of 4 low grade gliomas (LGGs) and targeted sequencing of 28 LGGs revealed ATRX variants are found exclusively in a subset of IDH1-mutant, 1p/19q intact LGGs. Paired blood sample… (full text at CIViC) PMID 23104868 · Kannan et al., 2012 · Open in CIViC	civic

ATRX Mutation4

(diagnostic)

Anaplastic AstrocytomaALIAS

Diagnostic

Supports Positive

submitted

EID8950

293 adult cases of low grade gliomas (LGGs) underwent a variety of analyses, including but not limited to exome sequencing (289 samples), copy number profiling (285), mRNA sequencing (277) and sequenc… (full text at CIViC)

PMID 26061751 · 2015, N. Engl. J. Med. · Open in CIViC

civic

〃

Brain GliomaUNRESOLVED

Diagnostic

Supports Positive

rejected

EID8866

The study examined 4 cases of low grade glioma (LGG) with whole exome sequencing, followed by targeted sequencing of 28 LGGs. The study demonstrated that all IDH-mutant LGG with intact 1p/19q status h… (full text at CIViC)

PMID 23104868 · Kannan et al., 2012 · Open in CIViC

civic

〃

Brain GliomaUNRESOLVED

Diagnostic

Supports Positive

rejected

EID8867

PMID 23104868 · Kannan et al., 2012 · Open in CIViC

civic

〃

Anaplastic AstrocytomaALIAS

Diagnostic

Supports Positive

accepted

EID8868

Whole exome sequencing of 4 low grade gliomas (LGGs) and targeted sequencing of 28 LGGs revealed ATRX variants are found exclusively in a subset of IDH1-mutant, 1p/19q intact LGGs. Paired blood sample… (full text at CIViC)

PMID 23104868 · Kannan et al., 2012 · Open in CIViC

civic

ATRX Underexpression4
(prognostic)	Childhood Low Grade Glioma	Prognostic	B	Supports Poor Outcome	4	accepted	EID7190 Loss of ATRX was identified in 12 of 247 patients with PLGGs using immunohistochemistry. Survival analysis showed that ATRX loss was associated with adverse overall survival (p<0.0001) and progression… (full text at CIViC) PMID 29948154 · Yang et al., 2018 · Open in CIViC	civic
〃	GlioblastomaCURATED_BROADER	Prognostic	D	Supports Poor Outcome	4	accepted	EID1648 In a glioblastoma mouse model induced by NRAS and p53 knockdown, ATRX loss was associated with a decreased median survival (69 days vs. 84 days; P = .0032). Also, the tumors grew to a larger size at e… (full text at CIViC) PMID 26936505 · Koschmann et al., 2016 · Open in CIViC	civic
PCV Regimen + TemozolomideSubstitutes	Anaplastic AstrocytomaALIAS	Predictive	B	Supports Sensitivity Response	3	accepted	EID1647 A retrospective tumor sample study found that the loss of ATRX expression (less than 10% of nuclei) in IDH-mutant astrocytomas treated with temozolomide or a combination of procarbazine, lomustine and… (full text at CIViC) PMID 23904111 · Wiestler et al., 2013 · Open in CIViC	civic
AZ20 + VE-821Substitutes	Osteosarcoma	Predictive	D	Supports Sensitivity Response	4	submitted	EID5832 Loss of the chromatin-remodeling protein ATRX associates with ALT in cancers. Inhibition of the protein kinase ATR, a critical regulator of recombination recruited by RPA, disrupts ALT and triggers ch… (full text at CIViC) PMID 25593184 · Flynn et al., 2015 · Open in CIViC	civic

ATRX Underexpression4

(prognostic)

Childhood Low Grade Glioma

Prognostic

Supports Poor Outcome

accepted

EID7190

Loss of ATRX was identified in 12 of 247 patients with PLGGs using immunohistochemistry. Survival analysis showed that ATRX loss was associated with adverse overall survival (p<0.0001) and progression… (full text at CIViC)

PMID 29948154 · Yang et al., 2018 · Open in CIViC

civic

〃

GlioblastomaCURATED_BROADER

Prognostic

Supports Poor Outcome

accepted

EID1648

In a glioblastoma mouse model induced by NRAS and p53 knockdown, ATRX loss was associated with a decreased median survival (69 days vs. 84 days; P = .0032). Also, the tumors grew to a larger size at e… (full text at CIViC)

PMID 26936505 · Koschmann et al., 2016 · Open in CIViC

civic

PCV Regimen + TemozolomideSubstitutes

Anaplastic AstrocytomaALIAS

Predictive

Supports Sensitivity Response

accepted

EID1647

A retrospective tumor sample study found that the loss of ATRX expression (less than 10% of nuclei) in IDH-mutant astrocytomas treated with temozolomide or a combination of procarbazine, lomustine and… (full text at CIViC)

PMID 23904111 · Wiestler et al., 2013 · Open in CIViC

civic

AZ20 + VE-821Substitutes

Osteosarcoma

Predictive

Supports Sensitivity Response

submitted

EID5832

Loss of the chromatin-remodeling protein ATRX associates with ALT in cancers. Inhibition of the protein kinase ATR, a critical regulator of recombination recruited by RPA, disrupts ALT and triggers ch… (full text at CIViC)

PMID 25593184 · Flynn et al., 2015 · Open in CIViC

civic