title: Use of murine bioassay to resolve ovine transmissible spongiform encephalopathy cases showing a bovine spongiform encephalopathy molecular profile.
abstract: Two cases of unusual transmissible spongiform encephalopathy (TSE) were diagnosed on the same farm in ARQ/ARQ PrP sheep showing attributes of both bovine spongiform encephalopathy (BSE) and scrapie. These cases, UK-1 and UK-2, were investigated further by transmissions to wild-type and ovine transgenic mice. Lesion profiles (LP) on primary isolation and subpassage, incubation period (IP) of disease, PrP(Sc)  immunohistochemical (IHC) deposition pattern and Western blot profiles were used to characterize the prions causing disease in these sheep. Results showed that both cases were compatible with scrapie. The presence of BSE was contraindicated by the following: LP on primary isolation in RIII and/or MR (modified RIII) mice; IP and LP after serial passage in wild-type mice; PrP(Sc)  deposition pattern in wild-type mice; and IP and Western blot data in transgenic mice. Furthermore, immunohistochemistry (IHC) revealed that each case generated two distinct PrP(Sc)  deposition patterns in both wild-type and transgenic mice, suggesting that two scrapie strains coexisted in the ovine hosts. Critically, these data confirmed the original differential IHC categorization that these UK-1 and UK-2 cases were not compatible with BSE.
authors.affiliation: Animal Health and Veterinary Laboratories Agency, Addlestone, Surrey, UK.
authors.forename: Katy E
authors.initials: KE
authors.lastname: Beck
authors.forename: Rosemary E
authors.initials: RE
authors.lastname: Sallis
authors.forename: Richard
authors.initials: R
authors.lastname: Lockey
authors.forename: Christopher M
authors.initials: CM
authors.lastname: Vickery
authors.forename: Vincent
authors.initials: V
authors.lastname: Béringue
authors.forename: Hubert
authors.initials: H
authors.lastname: Laude
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Holder
authors.forename: Leigh
authors.initials: L
authors.lastname: Thorne
authors.forename: Linda A
authors.initials: LA
authors.lastname: Terry
authors.forename: Anna C
authors.initials: AC
authors.lastname: Tout
authors.forename: Dhanushka
authors.initials: D
authors.lastname: Jayasena
authors.forename: Peter C
authors.initials: PC
authors.lastname: Griffiths
authors.forename: Saira
authors.initials: S
authors.lastname: Cawthraw
authors.forename: Richard
authors.initials: R
authors.lastname: Ellis
authors.forename: Anne
authors.initials: A
authors.lastname: Balkema-Buschmann
authors.forename: Martin H
authors.initials: MH
authors.lastname: Groschup
authors.forename: Marion M
authors.initials: MM
authors.lastname: Simmons
authors.forename: John
authors.initials: J
authors.lastname: Spiropoulos
mesh_terms: D000818:Animals; D001681:Biological Assay; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D002417:Cattle; D003937:Diagnosis, Differential; D016643:Encephalopathy, Bovine Spongiform / Q000175:diagnosis* / Q000473:pathology; D051379:Mice; D008822:Mice, Transgenic; D018620:PrPSc Proteins / Q000235:genetics / Q000378:metabolism*; D012608:Scrapie / Q000175:diagnosis* / Q000473:pathology; D012756:Sheep
chemical_list: D018620:PrPSc Proteins