Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

ATM Protein

  • Yosef Shiloh
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_435-3

Synonyms

Definition

ATM is a large, homeostatic protein kinase with roles in various branches of cellular metabolism. ATM’s best characterized function is mobilizing a highly complex network of signaling pathways in response to double-strand breaks (DSBs) in the DNA. DSBs markedly enhance the protein kinase activity of ATM, which subsequently phosphorylates a multitude of substrates, typically key players in the numerous branches of the DNA damage response. ATM is missing or inactive in patients with the multisystem genetic disorder, ataxia telangiectasia (A-T), characterized by neurodegeneration, immune deficiency, genomic instability, sensitivity to ionizing radiation, and cancer predisposition. The cellular phenotype of cells from A-T patients includes premature senescence, chromosomal instability, extreme sensitivity to DSB-inducing agents such as ionizing radiation and radiomimetic chemicals, and defective activation of the extensive DSB...

Keywords

Mantle Cell Lymphoma Meiotic Recombination Ataxia Telangiectasia Ataxia Telangiectasia Genotoxic Stress 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Paull TT (2015) Mechanisms of ATM activation. Ann Rev Biochem 84:711–738Google Scholar
  2. Shiloh Y (2014) ATM: expanding roles as a chief guardian of genome stability. Exp Cell Res 329:154–161CrossRefPubMedGoogle Scholar
  3. Shiloh Y, Ziv Y (2013) The ATM protein kinase: regulating the cellular response to genotoxic stress, and more. Nat Rev Cel Mol Biol 14:197–210CrossRefGoogle Scholar
  4. Thompson LH (2012) Recognition, signaling, and repair of DNA double-strand breaks produced by ionizing radiation in mammalian cells: the molecular choreography. Mutat Res 751:158–246CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Sackler School of MedicineTel Aviv UniversityTel AvivIsrael