Ataxia-telangiectasis (ATM) is a rare genetic neurodegenerative disease. About 20% of people with ATM develop acute lymphocytic leukemia or lymphoma, cancers of the immune-system cells. Cells in many of these cancers exhibit chromosome rearrangements, with chromosome breaks occurring at antibody and T-cell-receptor genes (A. L. Bredemeyer et al. 2006. Nature 442:466–470). Many people with ATM also have a weakened immune system, which makes them susceptible to respiratory infections. Research has shown that the locus that causes ATM has a role in the repair of double-strand breaks. Explain why people who have a genetic defect in the repair of doublestrand breaks might have a high incidence of chromosome rearrangements in their immune-system cells and why their immune systems might be weakened.

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