Open AccessUse of Murine Bioassay to Resolve Ovine Transmissible Spongiform Encephalopathy Cases Showing a Bovine Spongiform Encephalopathy Molecular Profile
Author(s) -
Beck Katy E.,
Sallis Rosemary E.,
Lockey Richard,
Vickery Christopher M.,
Béringue Vincent,
Laude Hubert,
Holder Thomas M.,
Thorne Leigh,
Terry Linda A.,
Tout Anna C.,
Jayasena Dhanushka,
Griffiths Peter C.,
Cawthraw Saira,
Ellis Richard,
BalkemaBuschmann Anne,
Groschup Martin H.,
Simmons Marion M.,
Spiropoulos John
Publication year - 2012
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/j.1750-3639.2011.00526.x
Subject(s) - bovine spongiform encephalopathy , scrapie , immunohistochemistry , virology , biology , transmissible spongiform encephalopathy , genetically modified mouse , western blot , lesion , pathology , microbiology and biotechnology , transgene , medicine , prion protein , disease , immunology , genetics , gene
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.