ABSTRACT
Multi-factorial information is available on Ataxia Telangiectasia (AT) phenotype associated with oxidative stress. First, the protein encoded by the AT gene (ATM) is involved in both the regulation of responses to double strand DNA breaks and of response to oxida tive stress. Moreover, an established collection of literature points to the roles of oxidative stress in AT that can be summarized as follows: (i) increased sensitivity of AT cells to DNA damaging agents via free radical mechanisms; (ii) increased sensitivity of AT cells to inflammatory cells and to ROS; (iii) protective effects of synthetic antioxidants in Atm knock-out mice; (iv) regulation of oxidative stress response in cell proliferation vs apoptosis, (v) abnormal levels of oxidative stress parameters in body fluids and blood cells from AT patients and (vi) impairment of mitochondrial functions and structure. Oxidative stress may thus be envisaged as a major phenomenon in AT s clinical phenotype.
