Molecular Classification
Protein Kinase, Serine/threonine kinase, PIKK superfamily
Disease Roles
Ataxia-telangiectasiaCancerNeurodegeneration

Ataxia Telangiectasia Mutated Protein Overview

ATM is a large serine/threonine protein kinase that plays a central role in the cellular response to DNA double-strand breaks (DSBs) and other forms of cellular stress. It is best known as the product of the gene mutated in ataxia-telangiectasia, a rare autosomal recessive disorder. ATM acts as a master regulator for signal transduction following DNA damage, phosphorylating key proteins and initiating cell cycle checkpoints. Loss-of-function mutations in ATM cause ataxia-telangiectasia. Targeted inhibition of ATM has been explored as a strategy for radiosensitizing tumors during cancer therapy.

Mechanism of Action

Inhibition of ATM kinase activity, blocking downstream signaling pathways involved in DNA damage repair and cell cycle arrest.

Biological Functions

DNA damage response
Cell cycle checkpoint control
DNA repair
Signal transduction
Transcriptional regulation
Translation control
Metabolism regulation
Apoptosis
Senescence

Disease Associations

Ataxia-telangiectasia
Cancer
Neurodegeneration
Immunodeficiency

Safety Considerations

  • Potential for off-target effects
  • Development of resistance
  • Toxicity
  • Inhibition of normal ATM function in healthy cells

Interacting Drugs

ATM inhibitors