First Author | Peter Y | Year | 2001 |
Journal | EMBO J | Volume | 20 |
Issue | 7 | Pages | 1538-46 |
PubMed ID | 11285218 | Mgi Jnum | J:68771 |
Mgi Id | MGI:1933435 | Doi | 10.1093/emboj/20.7.1538 |
Citation | Peter Y, et al. (2001) Elevated Cu/Zn-SOD exacerbates radiation sensitivity and hematopoietic abnormalities of Atm-deficient mice. EMBO J 20(7):1538-46 |
abstractText | Patients with the genetic disorder ataxia-telangiectasia (A-T) display a pleiotropic phenotype that includes neurodegeneration, immunodeficiency, cancer predisposition and hypersensitivity to ionizing radiation. The gene responsible is ATM, and ATM:-knockout mice recapitulate most features of A-T. In order to study the involvement of oxidative stress in the A-T phenotype, we examined mice deficient for Atm and overexpressing human Cu/Zn superoxide dismutase (SOD1). We report that elevated levels of SOD1 exacerbate specific features of the murine Atm- deficient phenotype, including abnormalities in hematopoiesis and radiosensitivity. The data are consistent with the possibility that oxidative stress contributes to some of the clinical features associated with the A-T phenotype. |