Reducing mitochondrial ROS improves disease-related pathology in a mouse model of ataxia-telangiectasia
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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|Source:||Molecular therapy : the journal of the American Society of Gene Therapy|
|01_D'Souza_Reducing_mitochondrial_ROS_2013.pdf||471.36 kB||Adobe PDF||Request a copy|
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