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Lecture in “Prions in Canada: Weird biology meets socioeconomic reality
February 2009

Neil R. Cashman
Canada Research Chair in Neurodegeneration and Protein Misfolding Diseases
Brain Research Centre
Department of Medicine (Neurology)
University of British Columbia
Director, ALS Centre GF Strong Hospital
Scientific Director, PrioNet Canada
tel: 604-222-3600


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Prion Diseases are fatal, transmissible diseases associated with a sponge-like degenerating of brain tissue in humans and animals. In animals, the most common of these diseases include scrapie (in sheep and goats), bovine spongiform encephalopathy (BSE in cattle), and chronic wasting disease (CWD in deer and elk). Some examples of prion diseases in humans include fatal familial insomnia and sporadic insomnia, Creutzfeldt-Jakob disease (CJD) and its many varieties in Kuru. Investigations continue into the potential link of prion diseases to other neurodegenerative disorders such as Alzheimer’s disease.

In May 2003, Canada’s cattle industry reeled when a confirmed case of BSE was discovered in Alberta. Overnight, the world’s borders closed to Canadian cattle. Canada’s economic loss stemming from this first BSE diagnosis is estimated at over $6 billion. The crisis left in its trail a heightened awareness about food safety, surveillance measures and human health concerns.

PrioNet was born out of the federal government‘s goal to overview the impacts caused by prion diseases in Canada. PrioNet Canada’s activities are developing strategies for a sustained, rational approach to mitigate, and ultimately control, prion diseases in Canada.




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