D'Souza, Anthony D; Parish, Ian; Krause, Diane S.; Kaech, Susan; Shadel, Gerald S
The disease ataxia-telangiectasia (A-T) has no cure and few treatment options. It is caused by mutations in the ATM kinase, which functions in the DNA-damage response and redox sensing. In addition to severe cerebellar degeneration, A-T pathology includes cancer predisposition, sterility, immune system dysfunction, and bone marrow abnormalities. These latter phenotypes are recapitulated in the ATM null (ATM-/-) mouse model of the disease. Since oxidative stress and mitochondrial dysfunction are...[Show more]
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