NR ATPK
AU Freixes,M.; Rodriguez,A.; Puig,B.; Ferrer,I.
TI Oxidative stress in Creutzfeldt-Jakob disease
QU International Conference - Prion 2005: Between fundamentals and society's needs - 19.10.-21.10.2005, Congress Center Düsseldorf - Poster Session: Pathogenesis PATH-21
PT Konferenz-Poster
AB The prion protein is the toxic agent of transmisible spongiform encephalopathies, also named prionopathies, which affect humans and animals. The prion has a N-terminal octarepeat region with cooper binding sites that may regulate the oxidative homeostasis. PrPc-/- mice show increased oxidation. The present study examines oxidative stress in the cerebral cortex and cerebellum in patients with sporadic Creutzfeldt-Jakob disease compared with age-matched controls. Increased expression of 4-hydroxynonenal (4-HNE) and malondialdehyde (MDA) has been observed in astrocytes and Purkinje cells. Preliminary results have also shown partially CML protease-resistant adducts in PrPsc-enriched fractions from CJD cases. Finally, indirect evidence suggest nitration of PrP in disease. These changes are associated with increased expression of SOD1. Together these findings indicate that oxidative estress may have implications in the pathogenesis of CJD.
AD M.Freixes, A.Rodríguez, B.Puig, I.Ferrer, Institut de Neuropatologia, Servei d'Anatomía Patològica, IDIBELL-Hospital Universitari de Bellvitge, Hospitalet de LLobregat, Spain; I.Ferrer, Unitat de Neuropatología Experimental, Universität de Barcelona, Hospitalet de LLobregat, Spain, 8082ifa@comb.es
SP englisch
PO Deutschland