title: Characterization of atypical scrapie cases from Great Britain in transgenic ovine PrP mice.
abstract: Twenty-four atypical scrapie cases from sheep with different prion protein genotypes from Great Britain were transmitted to transgenic tg338 and/or TgshpXI mice expressing sheep PrP alleles, but failed to transmit to wild-type mice. Mean incubation periods were 200-300 days in tg338 mice and 300-500 days in TgshpXI mice. Survival times in C57BL/6 and VM/Dk mice were >700 days. Western blot analysis of mouse brain samples revealed similar multi-band, protease-resistant prion protein (PrP(res)) profiles, including an unglycosylated band at approximately 8-11 kDa, which was shown by antibody mapping to correspond to the approximately 93-148 aa portion of the PrP molecule. In transgenic mice, the incubation periods, Western blot PrP(res) profiles, brain lesion profiles and abnormal PrP (PrP(Sc)) distribution patterns produced by the Great Britain atypical scrapie isolates were similar and compatible with the biological characteristics of other European atypical scrapie or Nor98 cases.
authors.affiliation: Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Peter C
authors.initials: PC
authors.lastname: Griffiths
authors.affiliation: Neuropathology Section, Pathology Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: John
authors.initials: J
authors.lastname: Spiropoulos
authors.affiliation: Neuropathology Section, Pathology Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Richard
authors.initials: R
authors.lastname: Lockey
authors.affiliation: Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Anna C
authors.initials: AC
authors.lastname: Tout
authors.affiliation: Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Dhanushka
authors.initials: D
authors.lastname: Jayasena
authors.affiliation: Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Jane M
authors.initials: JM
authors.lastname: Plater
authors.affiliation: Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Alun
authors.initials: A
authors.lastname: Chave
authors.affiliation: Neuropathology Section, Pathology Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Robert B
authors.initials: RB
authors.lastname: Green
authors.affiliation: Neuropathology Section, Pathology Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Sarah
authors.initials: S
authors.lastname: Simonini
authors.affiliation: Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Leigh
authors.initials: L
authors.lastname: Thorne
authors.affiliation: Animal Services Unit, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Ian
authors.initials: I
authors.lastname: Dexter
authors.affiliation: Friedrich-Loeffler-Institut, Federal Research Institute for Animal Health, 17493 Greifswald-Insel Riems, Germany.
authors.forename: Anne
authors.initials: A
authors.lastname: Balkema-Buschmann
authors.affiliation: Friedrich-Loeffler-Institut, Federal Research Institute for Animal Health, 17493 Greifswald-Insel Riems, Germany.
authors.forename: Martin H
authors.initials: MH
authors.lastname: Groschup
authors.affiliation: Virologie Immunologie Moléculaires, U892, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Béringue
authors.affiliation: Virologie Immunologie Moléculaires, U892, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France.
authors.forename: Annick
authors.initials: A
authors.lastname: Le Dur
authors.affiliation: Virologie Immunologie Moléculaires, U892, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France.
authors.forename: Hubert
authors.initials: H
authors.lastname: Laude
authors.affiliation: Centre for Epidemiology and Risk Analysis, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: James
authors.initials: J
authors.lastname: Hope
mesh_terms: D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000473:pathology; D006651:Histocytochemistry; D007150:Immunohistochemistry; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D008970:Molecular Weight; D011328:Prions / Q000737:chemistry / Q000302:isolation & purification; D012608:Scrapie / Q000635:transmission*; D012756:Sheep; D012757:Sheep Diseases / Q000635:transmission*; D016019:Survival Analysis; D006113:United Kingdom
chemical_list: D011328:Prions