Transmissible spongiform encephalopathy disease of humans is generally sporadic or genetic in nature, however, disease can also be acquired, possibly by consumption of infected food. It is therefore possible that further animal TSEs may emerge which are capable of infecting humans. In particular, atypical forms of sheep scrapie and BSE have been recently identified in Europe. The risk of infection and spread of these agents within the human population must be identified in order to prevent further human disease.
To assess the risk to humans of infection from cases of atypical scrapie, humanised transgenic mice will be inoculated intracerebrally with isolates of atypical sheep scrapie and monitored for signs of TSE disease. This will be used to assess the susceptibility of humans to atypical scrapie. Similarly, the risk to humans of infection from chronic wasting disease (a disease in deer) and atypical forms of BSE will be investigated. Mice will be analysed by vacuolation profiling and immunohistochemistry to identify and characterise TSE associated pathology in the brain. Human to human transmission of disease will be further investigated by subpassage (secondary transmission within the same species) of the clinical cases.
1. Wilson, R., Hart, P., Piccardo, P., Hunter, N., Casalone, C., Baron, T. & Barron, R., (2012) Bovine PrP expression levels in transgenic mice influence transmission characteristics of atypical BSE. Journal of General Virology 93(5), 1132-40
2. 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. & Barron, R. (2012) Chronic wasting disease and atypical forms of BSE and scrapie are not transmissible to mice expressing wild-type levels of human PrP. Journal of General Virology 93(7),1624-9