NR ATMU
AU Michaud,D.; Privat,N.; Laffont-Proust,I.; Sazdovitch,V.; Peoc'h,K.; Laplanche,J.L.; Faucheux,B.A.; Hauw,J.J.; Haik,S.
TI PrPsc deposit in ten different prion diseases : an immmunihistochemical study using a panel of monoclonal antibodies
QU International Conference - Prion 2005: Between fundamentals and society's needs - 19.10.-21.10.2005, Congress Center Düsseldorf - Poster Session: Genetics, strains and emerging problems GEN-20
PT Konferenz-Poster
AB Inherited TSEs are rare (only ten percent of all human cases) but highly heterogeneous diseases. More than fifty-five mutations have been described in the PRNP coding sequence. This leads to sparse reports of single cases or families. Here, we propose a comprehensive description of prion protein accumulation pattern in the brain of 18 patients with ten different inherited TSEs (one insertion within the octarepeat domain, mutations: P102L, D178N-129M, D178N-129V, V180I, E196K, E200K-129M, E200K-129V, V203I and R208H). These mutations were differently associated to various clinico-pathological phenotypes: Creutzfeldt-Jakob disease, fatal insomnia and Gerstmann-Sträussler-Scheinker syndrome. PrPsc deposits were studied by immunohistochemistry using a panel of 5 monoclonal antibodies. The antibodies used recognize different linear epitopes that are dispatched among PrP sequence. Different brain regions were studied including frontal and occipital isocortices, head of the caudate nucleus, thalamus and cerebellum. Results of this study provide comparisons between i) the PrPsc deposit pattern between inherited diseases, ii) recognized epitopes and iii) prion targeted brain regions.
AD Dorothée Michaud, Nicolas Privat, Isabelle Laffont-Proust, Véronique Sazdovitch, Baptiste A. Faucheux, Jean-Jacques Hauw, Stéphane Haïk, INSERM Avenir Team - Human Prion Diseases, IFR70, Neuropathology, Salpêtrière Hospital, Paris, France; Dorothée Michaud, Université de Montréal, Montréal, Canada; Katell Peoc'h, Jean-Louis Laplanche, UPRES EA 3621, UFR des Sciences Pharmaceutiques et Biologiques, Université de Paris 5 et service de Biochimie et Biologie Moléculaire, Hôpital Lariboisière, Paris, France
SP englisch
PO Deutschland