Prion Transmission

Prion-caused wasting

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Science  03 Apr 2015:
Vol. 348, Issue 6230, pp. 88
DOI: 10.1126/science.348.6230.88-a

Unique prions cause chronic wasting disease in North American elk and deer


Chronic wasting disease (CWD) is killing deer and elk in the United States and Canada. CWD is caused by an infectious protein, or prion, that is found in meat and blood from infected animals. But is there a risk to humans who eat these animals? Kurt et al. found that mice expressing human prion protein (PrP) resisted CWD, whereas mice expressing human PrP with four elk residue substitutions were susceptible. Only two elk residue substitutions in human PrP were needed for efficient conversion of the protein to the prion conformation. The amyloid-forming propensity of the PrP was important in this conversion, which may explain why some species are highly susceptible to prions from other species.

J. Clin. Invest. 10.1172/JCI79408 (2015).

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