Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein
Date
2012-07-01Author
Wilson, Rona
Plinston, Chris
Hunter, Nora
Casalone, Cristina
Corona, Cristiano
Tagliavini, Fabrizio
Suardi, SIlvia
Ruggerone, Margherita
Graziano, Silvia
Sbriccoli, Marco
Cardone, Franco
Pocchiari, Maurizio
Ingrosso, Loredana
Baron, Thierry
Richt, Juergen
Andreoletti, Olivier
Simmons, Marion
Lockey, Richard
Manson, Jean C.
Barron, Rona
Metadata
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Wilson, R., Plinston, C., Hunter, N., Casalone, C., Corona, C., Tagliavini, F., Suardi, S., Ruggerone, M., Moda, F., Graziano, S., Sbriccoli, M., Cardone, F., Pocchiari, M., Ingrosso, L., Baron, T., Richt, J., Andreoletti, O., Simmons, M., Lockey, R., Manson, J.C. and Barron, R.M. (2012) ‘Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein’, Journal of General Virology, 93(7), pp. 1624–1629. Available at: https://doi.org/10.1099/vir.0.042507-0.
Abstract
The association between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt–Jakob disease (vCJD) has demonstrated that cattle transmissible spongiform encephalopathies (TSEs) can pose a risk to human health and raises the possibility that other ruminant TSEs may be transmissible to humans. In recent years, several novel TSEs in sheep, cattle and deer have been described and the risk posed to humans by these agents is currently unknown. In this study, we inoculated two forms of atypical BSE (BASE and H-type BSE), a chronic wasting disease (CWD) isolate and seven isolates of atypical scrapie into gene-targeted transgenic (Tg) mice expressing the human prion protein (PrP). Upon challenge with these ruminant TSEs, gene-targeted Tg mice expressing human PrP did not show any signs of disease pathology. These data strongly suggest the presence of a substantial transmission barrier between these recently identified ruminant TSEs and humans.