title: Atypical prion protein in sheep brain collected during the British scrapie-surveillance programme.
abstract: Scrapie of sheep and goats is the most common prion disease (or transmissible spongiform encephalopathy, TSE) of mammals and aggregates of abnormal, proteinase-resistant prion protein (PrP(Sc)) are found in all naturally occurring prion diseases. During active surveillance of British sheep for TSEs, 29 201 sheep brain stem samples were collected from abattoirs and analysed for the presence of PrP(Sc). Of these samples, 54 were found to be positive by using an ELISA screening test, but 28 of these could not be confirmed initially by immunohistochemistry. These unconfirmed or atypical cases were generally found in PrP genotypes normally associated with relative resistance to clinical scrapie and further biochemical analysis revealed that they contained forms of PrP(Sc) with a relatively protease-sensitive amyloid core, some resembling those of Nor98 scrapie. The presence of these atypical forms of protease-resistant PrP raises concerns that some TSE disorders of PrP metabolism previously may have escaped identification in the British sheep population.
authors.affiliation: Department of TSE Molecular Biology, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey KT15 3NB, UK.
authors.forename: S J
authors.initials: SJ
authors.lastname: Everest
authors.affiliation: Department of TSE Molecular Biology, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey KT15 3NB, UK.
authors.forename: L
authors.initials: L
authors.lastname: Thorne
authors.affiliation: Department of TSE Molecular Biology, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey KT15 3NB, UK.
authors.forename: D A
authors.initials: DA
authors.lastname: Barnicle
authors.affiliation: Department of TSE Molecular Biology, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey KT15 3NB, UK.
authors.forename: J C
authors.initials: JC
authors.lastname: Edwards
authors.affiliation: Institute for Animal Health, Pirbright Laboratory, Woking, Surrey, UK.
authors.forename: H
authors.initials: H
authors.lastname: Elliott
authors.affiliation: Department of TSE Molecular Biology, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey KT15 3NB, UK.
authors.forename: R
authors.initials: R
authors.lastname: Jackman
authors.affiliation: Veterinary Laboratories Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Midlothian EH26 0PZ, UK.
authors.forename: J
authors.initials: J
authors.lastname: Hope
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology; D007150:Immunohistochemistry / Q000662:veterinary; D018620:PrPSc Proteins / Q000032:analysis*; D011328:Prions / Q000302:isolation & purification*; D012608:Scrapie / Q000453:epidemiology / Q000276:immunology / Q000378:metabolism* / Q000635:transmission; D012756:Sheep; D006113:United Kingdom / Q000453:epidemiology
chemical_list: D018620:PrPSc Proteins; D011328:Prions