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ATM ATM serine/threonine kinase

Gene ID: 472, updated on 12-Apr-2026
Gene type: protein coding
Also known as: AT1; ATA; ATC; ATD; ATE; ATDC; TEL1; TELO1

Summary

The protein encoded by this gene belongs to the PI3/PI4-kinase family. This protein is an important cell cycle checkpoint kinase that phosphorylates; thus, it functions as a regulator of a wide variety of downstream proteins, including tumor suppressor proteins p53 and BRCA1, checkpoint kinase CHK2, checkpoint proteins RAD17 and RAD9, and DNA repair protein NBS1. This protein and the closely related kinase ATR are thought to be master controllers of cell cycle checkpoint signaling pathways that are required for cell response to DNA damage and for genome stability. Mutations in this gene are associated with ataxia telangiectasia, an autosomal recessive disorder. [provided by RefSeq, Aug 2010]

Associated conditions

See all available tests in GTR for this gene

DescriptionTests
Ataxia-telangiectasia syndromeSee labs
Common variants near ATM are associated with glycemic response to metformin in type 2 diabetes.
GeneReviews: Not available
Does metformin work for everyone? A genome-wide association study for metformin response.
GeneReviews: Not available
Familial cancer of breastSee labs
Genome-wide association study identifies three new melanoma susceptibility loci.
GeneReviews: Not available

Copy number response

Description
Copy number response
Triplosensitivity

No evidence available (Last evaluated 2020-04-08)

ClinGen Genome Curation Page
Haploinsufficency

Sufficient evidence for dosage pathogenicity (Last evaluated 2020-04-08)

ClinGen Genome Curation PagePubMed

Genomic context

Location:
11q22.3
Sequence:
Chromosome: 11; NC_000011.10 (108223067..108369102)
Total number of exons:
67

Links

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