Prion Strain Mutation and Selection

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Science  28 May 2010:
Vol. 328, Issue 5982, pp. 1111-1112
DOI: 10.1126/science.1190815

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When the connection was made between bovine spongiform encephalopathy (BSE or “mad cow” disease) and human illnesses in the 1990s, it raised the public profile of the underlying prion diseases as the implications of animal and public health crises, and their economic impacts, became apparent. At the core of these concerns is how prions, the infectious agent, diversify and expand their host range. On page 1154 of this issue, Angers et al. (1) reveal how this occurs in chronic wasting disease (CWD), a contagious prion disease of wild deer and elk. Its prevalence in the United States raised fears that, like BSE, it might transmit to humans.