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Reducing mitochondrial ROS improves disease-related pathology in a mouse model of ataxia-telangiectasia

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]

CollectionsANU Research Publications
Date published: 2013
Type: Journal article
Source: Molecular therapy : the journal of the American Society of Gene Therapy
DOI: 10.1038/mt.2012.203


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