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Originally published in Science Express on 26 January 2006
Science 24 February 2006:
Vol. 311. no. 5764, p. 1117
DOI: 10.1126/science.1122864

Brevia

Prions in Skeletal Muscles of Deer with Chronic Wasting Disease

Rachel C. Angers,1* Shawn R. Browning,1*{dagger} Tanya S. Seward,2 Christina J. Sigurdson,4{ddagger} Michael W. Miller,5 Edward A. Hoover,4 Glenn C. Telling1,2,3§

The emergence of chronic wasting disease (CWD) in deer and elk in an increasingly wide geographic area, as well as the interspecies transmission of bovine spongiform encephalopathy to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about the zoonotic potential of CWD. Because meat consumption is the most likely means of exposure, it is important to determine whether skeletal muscle of diseased cervids contains prion infectivity. Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.

1 Department of Microbiology, Immunology and Molecular Genetics, University of Kentucky, Lexington, KY 40536, USA.
2 Sanders Brown Center on Aging, University of Kentucky, Lexington, KY 40536, USA.
3 Department of Neurology, University of Kentucky, Lexington, KY 40536, USA.
4 Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA.
5 Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA.

* These authors contributed equally to this work.

{dagger} Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, FL 33458, USA.

{ddagger} Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland.

§ To whom correspondence should be addressed. E-mail: gtell2{at}uky.edu

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Science. ISSN 0036-8075 (print), 1095-9203 (online)