title: PrP(d) accumulation in organs of ARQ/ARQ sheep experimentally infected with BSE by peripheral routes.
abstract: To study the pathogenesis of bovine spongiform encephalopathy infection in small ruminants, two Lacaune sheep with the AA136RR154QQ171 and one with the AA136RR154RR171 genotype for the prion protein, were inoculated with a brain homogenate from a French cattle BSE case by peripheral routes. Sheep with the ARQ/ARQ genotype are considered as susceptible to prion diseases contrary to those with the ARR/ARR genotype. The accumulation of disease-associated prion protein (PrP(d)) was analysed by biochemical and immunohistochemical methods. No PrP(d) accumulation was detected in samples from the ARR/ARR sheep 2 years post inoculation. In the two ARQ/ARQ sheep that had scrapie-like clinical symptoms, PrP(d) was found in the central, sympathetic and enteric nervous systems and in lymphoid organs. Remarkably, PrP(d) was also detected in some muscle types as well as in all peripheral nerves that had not been reported previously thus revealing a widespread distribution of BSE-associated PrP(d) in sheep tissues.
authors.affiliation: Agence Française de Sécurité Sanitaire des Aliments (AFSSA), Lyon cedex 07, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Lezmi
authors.forename: Frédéric
authors.initials: F
authors.lastname: Ronzon
authors.forename: Anna
authors.initials: A
authors.lastname: Bencsik
authors.forename: Alexandre
authors.initials: A
authors.lastname: Bedin
authors.forename: Didier
authors.initials: D
authors.lastname: Calavas
authors.forename: Yves
authors.initials: Y
authors.lastname: Richard
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Simon
authors.forename: Jacques
authors.initials: J
authors.lastname: Grassi
authors.forename: Thierry
authors.initials: T
authors.lastname: Baron
mesh_terms: D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002417:Cattle; D002490:Central Nervous System / Q000378:metabolism / Q000473:pathology; D004064:Digestive System / Q000378:metabolism / Q000473:pathology; D016643:Encephalopathy, Bovine Spongiform / Q000378:metabolism* / Q000473:pathology; D005838:Genotype; D007150:Immunohistochemistry; D008208:Lymphatic System / Q000378:metabolism / Q000473:pathology; D018620:PrPSc Proteins / Q000032:analysis* / Q000235:genetics; D012160:Retina / Q000378:metabolism / Q000473:pathology; D012608:Scrapie / Q000378:metabolism / Q000473:pathology; D012756:Sheep
chemical_list: D018620:PrPSc Proteins