Characterization of atypical scrapie cases from Great Britain in transgenic ovine PrP mice.
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.
Griffiths
Peter C
PC
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Spiropoulos
John
J
Neuropathology Section, Pathology Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Lockey
Richard
R
Neuropathology Section, Pathology Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Tout
Anna C
AC
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Jayasena
Dhanushka
D
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Plater
Jane M
JM
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Chave
Alun
A
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Green
Robert B
RB
Neuropathology Section, Pathology Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Simonini
Sarah
S
Neuropathology Section, Pathology Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Thorne
Leigh
L
Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Dexter
Ian
I
Animal Services Unit, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
Balkema-Buschmann
Anne
A
Friedrich-Loeffler-Institut, Federal Research Institute for Animal Health, 17493 Greifswald-Insel Riems, Germany.
Groschup
Martin H
MH
Friedrich-Loeffler-Institut, Federal Research Institute for Animal Health, 17493 Greifswald-Insel Riems, Germany.
Béringue
Vincent
V
Virologie Immunologie Moléculaires, U892, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France.
Le Dur
Annick
A
Virologie Immunologie Moléculaires, U892, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France.
Laude
Hubert
H
Virologie Immunologie Moléculaires, U892, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France.
Hope
James
J
Centre for Epidemiology and Risk Analysis, Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
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
D011328:Prions