NR AEXX
AU Guentchev,M.; Siedlak,S.L.; Jarius,C.; Tagliavini,F.; Castellani,R.J.; Perry,G.; Smith,M.A.; Budka,H.
TI Oxidative damage to nucleic acids in human prion disease
QU Neurobiology of Disease 2002 Apr; 9(3): 275-81
PT journal article
AB Recently, several studies proposed a physiological role for the cellular prion protein (PrPc) in defense against oxidative stress. Since the pathogenesis of prion disease necessarily involves a disturbance of PrPc homeostasis, we hypothesized that such diseases would be associated with concomitant disturbances in oxidative balance. In support of such a notion, in this study we show increased oxidative damage to nucleic acids in affected brains of patients with Creutzfeldt-Jakob disease. These data suggest that damage by free radicals is a likely cause for neurodegeneration in human prion disease, and antioxidants are a potential therapy for these disorders. Further, our data support the hypothesis that loss of the anti-oxidant function of PrPc plays a key role in the pathogenesis of these disorders.
MH Adult; Aged; Brain/metabolism/pathology; Brain Chemistry; Comparative Study; Female; Human; Male; Middle Age; Nucleic Acids/*analysis/biosynthesis; *Oxidative Stress/physiology; Prion Diseases/*metabolism/*pathology; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Institute of Neurology, University of Vienna, Austrian Reference Center for Human Prion Disease (ORPE).
SP englisch
PO USA