Oxidative-stress-induced apoptosis in PBLs of two patients with Parkinson disease secondary to alpha-synuclein mutation.
Alpha-synuclein has been implicated in the pathology of certain neurodegenerative diseases, including Parkinson disease (PD). Although the precise physiological and pathological role of alpha-synuclein is unclear, overexpression of the protein or its mutants may reduce cell viability. In this study we evaluated the apoptotic response to oxidative stress induced by 2-deoxy-d-ribose (dRib) in peripheral blood lymphocytes (PBLs) of two siblings with Parkinson disease secondary to A53T alpha-synuclein mutation. PBLs exposed to oxidative stress showed a higher percentage of apoptotic cells in PD patients than in controls. However in cells of PD patients, the increase of apoptotic response was lower than in controls, suggesting that cells of PD patients have greater "resistance" to oxidative stress. We conclude that other environmental agents could play a key role in inducing programmed cell death in cells of PD patients with mutant alpha-synuclein.
Department of Neurological and Behavioural Sciences, O.U. of Neurometabolic Disease, University of Siena, Siena, Italy.
Carla
C
Battisti
Patrizia
P
Formichi
Elena
E
Radi
Antonio
A
Federico
D000328:Adult; D017209:Apoptosis / Q000187:drug effects / Q000235:genetics*; D004252:DNA Mutational Analysis; D003855:Deoxyribose / Q000633:toxicity; D004777:Environment; D005434:Flow Cytometry; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005838:Genotype; D006801:Humans; D007113:Immunity, Innate / Q000187:drug effects / Q000235:genetics; D008214:Lymphocytes / Q000378:metabolism* / Q000473:pathology; D008297:Male; D009154:Mutation / Q000235:genetics; D018384:Oxidative Stress / Q000187:drug effects / Q000235:genetics*; D010300:Parkinson Disease / Q000097:blood* / Q000235:genetics* / Q000503:physiopathology; D051844:alpha-Synuclein / Q000235:genetics*
D051844:alpha-Synuclein; D003855:Deoxyribose