Damage Control Team

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Science  02 Jun 2000:
Vol. 288, Issue 5471, pp. 1551
DOI: 10.1126/science.288.5471.1551c

Patients with the rare inherited diseases ataxia telangiectasia (AT) and Nijmegen breakage syndrome (NBS) present a similar profile of cellular symptoms, including radiation sensitivity and chromosome breakage, which is reflected clinically as an enhanced susceptibility to cancer. Four independent laboratories have converged on the reason for the similarity in cellular phenotype: the defective proteins in the two disorders (ATM, a protein kinase, and NBS1, a DNA repair protein) function in a common pathway for sensing and repairing a specific type of DNA damage. Their results reveal that when double-strand DNA breaks occur, ATM phosphorylates NBS1, and this phosphorylation is required for NBS1 to prevent damaged DNA from being copied. Thus, the integrity of the genome depends on the concerted action of ATM and NBS1, with loss of either protein producing the same devastating consequences.—PAK

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