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: Jonathan D F Wadsworth, Susan Joiner, Jacqueline M Linehan, Anne Balkema-Buschmann, John Spiropoulos, Marion M Simmons, Peter C Griffiths, Martin H Groschup, James Hope, Sebastian Brandner, Emmanuel A Asante, John Collinge
• Journal: Emerging infectious diseases (Emerg Infect Dis) Vol. 19 Issue 11 Pg. 1731-9 (Nov 2013) ISSN: 1080-6059 [Electronic] United States
• PMID: 24188521 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Prions

Topics

• Animals
• Brain (metabolism, pathology)
• Cattle
• Encephalopathy, Bovine Spongiform (metabolism, pathology)
• Gene Expression
• Humans
• Mice
• Mice, Transgenic
• Prions (genetics, metabolism)
• Scrapie (genetics, transmission)
• Sheep
• Species Specificity