Gene Information

CHEK2

checkpoint kinase 2 [Source:HGNC Symbol;Acc:HGNC:16627]

protein_codingChromosome 22ENSG00000183765

Overview

CHEK2 variants are associated with 5 diseases including bone osteosarcoma and Li-Fraumeni syndrome and 3 others, with 12 documented clinical phenotypes.


Associated Diseases

The CHEK2 gene is associated with 5 medical conditions, including:

bone osteosarcoma

MONDO:0002629

Li-Fraumeni syndrome

MONDO:0018875

hereditary breast ovarian cancer syndrome

MONDO:0003582

prostate cancer, hereditary

MONDO:0700275

familial colorectal cancer type X

MONDO:0018604


Clinical Phenotypes

Research has identified 12 phenotypic features associated with CHEK2 variants:

Prostate cancer

HP:0012125

Sarcoma

HP:0100242

Osteosarcoma

HP:0002669

Retinoblastoma

HP:0009919

Typified by somatic mosaicism

HP:0001442

Meningioma

HP:0002858

Autosomal dominant inheritance

HP:0000006

Breast carcinoma

HP:0003002

Autosomal dominant inheritance

HP:0000006

Stomach cancer

HP:0012126

Glioma

HP:0009733

Typified by somatic mosaicism

HP:0001442


ClinVar Variants

ClinVar contains 10 variants associated with CHEK2. Below are some notable variants:

NM_007194.4(CHEK2):c.1312del (p.Asp438fs)

Pathogenic

VCV004294199

Review: criteria provided, single submitter

NM_007194.4(CHEK2):c.969del (p.Cys324fs)

Pathogenic

VCV004294163

Review: criteria provided, single submitter

NM_007194.4(CHEK2):c.684-86A>G

Likely benign

VCV004280722

Review: criteria provided, single submitter

GRCh37/hg19 22q12.1(chr22:28941617-29145805)x1

Pathogenic

VCV004279335

Review: criteria provided, single submitter

NM_007194.4(CHEK2):c.1009-87del

Likely pathogenic

VCV004279034

Review: criteria provided, single submitter


Recent Publications

There are 1,125 publications about CHEK2 indexed in PubMed. Below are some recent papers:

Beyond the nucleus: the ATM-CHEK2 axis senses mtROS to orchestrate Mitophagy.

Guo QQ, Song XY, Cao L

PMID: 41257485

2025 Nov 19

Autophagy

Evaluating the Role of Liquid Biopsy to Detect Pathogenic Homologous Recombination Repair (HRR) Gene Alterations in Metastatic Prostate Cancer.

Labidi S, Jiao B, Tam S

PMID: 41228221

2025 Oct 25

Cancers (Basel)

Methionine deficiency causes spermatogonial apoptosis via oxidative stress and DNA damage response pathway.

Wang W, Ruan Y, Ting G

PMID: 41219782

2025 Nov 12

Biol Res

Integrating Pathogenic Variants, Polygenic Risk Score, and Family History for Prostate Cancer Risk Estimation in Men of African Ancestry.

Chen F, Sheng X, Wang A

PMID: 41219045

2025 Nov 5

Eur Urol

CHEK2 variants, breast cancer, and implications for management: a narrative review.

Conde DM

PMID: 41210647

2025

Transl Breast Cancer Res

View all 1,125 publications on PubMed →

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Trusted Data Sources

EnsemblMonarch InitiativeClinVarGeneReviewsPubMed

Last updated: 11/20/2025

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