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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...
References
Paull TT (2015) Mechanisms of ATM activation. Ann Rev Biochem 84:711–738
Shiloh Y (2014) ATM: expanding roles as a chief guardian of genome stability. Exp Cell Res 329:154–161
Shiloh Y, Ziv Y (2013) The ATM protein kinase: regulating the cellular response to genotoxic stress, and more. Nat Rev Cel Mol Biol 14:197–210
Thompson LH (2012) Recognition, signaling, and repair of DNA double-strand breaks produced by ionizing radiation in mammalian cells: the molecular choreography. Mutat Res 751:158–246
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Shiloh, Y. (2014). ATM Protein. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_435-3
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DOI: https://doi.org/10.1007/978-3-642-27841-9_435-3
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Publisher Name: Springer, Berlin, Heidelberg
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