Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein

Wadsworth, Jonathan D.F.; Joiner, Susan; Linehan, Jacqueline M.; Balkema-Buschmann, Anne; Spiropoulos, John; Simmons, Marion M.; Griffiths, Peter C.; Groschup, Martin H.; Hope, James; Brandner, Sebastian; Asante, Emmanuel A.; Collinge, John

doi:10.3201/eid1911.121341

Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein

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Wadsworth, Jonathan D.F., Joiner, Susan, Linehan, Jacqueline M., Balkema-Buschmann, Anne, Spiropoulos, John, Simmons, Marion M., Griffiths, Peter C., Groschup, Martin H., Hope, James, Brandner, Sebastian, Asante, Emmanuel A. and Collinge, John (2013) Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein. Emerging Infectious Diseases, 19 (11). pp. 1731-1739. ISSN 1080-6040

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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.

Item Type:	Article
Uncontrolled Keywords:	epidemiology,microbiology (medical),infectious diseases,sdg 3 - good health and well-being ,/dk/atira/pure/subjectarea/asjc/2700/2713
Faculty \ School:	Faculty of Science > School of Biological Sciences
Related URLs:	http://www.scopus.com/inward/record.url?...
Depositing User:	LivePure Connector
Date Deposited:	04 Dec 2025 12:30
Last Modified:	08 Dec 2025 12:30
URI:	https://ueaeprints.uea.ac.uk/id/eprint/101253
DOI:	10.3201/eid1911.121341

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