title: Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein.
abstract: Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in ruminants may also have zoonotic potential. One example is atypical/Nor98 scrapie, which evaded statutory diagnostic methods worldwide until the early 2000s. To investigate whether sheep infected with scrapie prions could be another source of infection, we inoculated transgenic mice that overexpressed human prion protein with brain tissue from sheep with natural field cases of classical and atypical scrapie, sheep with experimental BSE, and cattle with BSE. We found that these mice were susceptible to BSE prions, but disease did not develop after prolonged postinoculation periods when mice were inoculated with classical or atypical scrapie prions. These data are consistent with the conclusion that prion disease is less likely to develop in humans after exposure to naturally occurring prions of sheep than after exposure to epizootic BSE prions of ruminants.
authors.forename: Jonathan D F
authors.initials: JD
authors.lastname: Wadsworth
authors.forename: Susan
authors.initials: S
authors.lastname: Joiner
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Linehan
authors.forename: Anne
authors.initials: A
authors.lastname: Balkema-Buschmann
authors.forename: John
authors.initials: J
authors.lastname: Spiropoulos
authors.forename: Marion M
authors.initials: MM
authors.lastname: Simmons
authors.forename: Peter C
authors.initials: PC
authors.lastname: Griffiths
authors.forename: Martin H
authors.initials: MH
authors.lastname: Groschup
authors.forename: James
authors.initials: J
authors.lastname: Hope
authors.forename: Sebastian
authors.initials: S
authors.lastname: Brandner
authors.forename: Emmanuel A
authors.initials: EA
authors.lastname: Asante
authors.forename: John
authors.initials: J
authors.lastname: Collinge
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000378:metabolism / Q000473:pathology; D015870:Gene Expression; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D011328:Prions / Q000235:genetics* / Q000378:metabolism; D012608:Scrapie / Q000235:genetics* / Q000635:transmission*; D012756:Sheep; D013045:Species Specificity
chemical_list: D011328:Prions
keywords: Atypical scrapie; BSE; Nor98 scrapie; bovine spongiform encephalopathy; prion disease; prion protein; prions; scrapie; sheep BSE; transgenic mice; transmissible spongiform encephalopathy (TSE); vCJD; variant Creutzfeldt-Jakob disease