TY - JOUR
T1 - Oxidative damage to nucleic acids in human prion disease
AU - Guentchev, Marin
AU - Siedlak, Sandra L.
AU - Jarius, Christa
AU - Tagliavini, Fabrizio
AU - Castellani, Rudy J.
AU - Perry, George
AU - Smith, Mark A.
AU - Budka, Herbert
PY - 2002
Y1 - 2002
N2 - 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.
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.
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U2 - 10.1006/nbdi.2002.0477
DO - 10.1006/nbdi.2002.0477
M3 - Article
C2 - 11950273
AN - SCOPUS:0036236406
VL - 9
SP - 275
EP - 281
JO - Neurobiology of Disease
JF - Neurobiology of Disease
SN - 0969-9961
IS - 3
ER -