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The Journal of Neuroscience, August 31, 2005, 25(35):7944-7949; doi:10.1523/JNEUROSCI.2467-05.2005

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Neurobiology of Disease
Chronic Wasting Disease of Elk: Transmissibility to Humans Examined by Transgenic Mouse Models

Qingzhong Kong,¹ Shenghai Huang,¹ Wenquan Zou,¹ Difernando Vanegas,¹ Meiling Wang,¹ Di Wu,¹ Jue Yuan,¹ Mengjie Zheng,¹ Hua Bai,¹ Huayun Deng,² Ken Chen,³ Allen L. Jenny,⁴ Katherine O'Rourke,⁵ Ermias D. Belay,⁶ Lawrence B. Schonberger,⁶ Robert B. Petersen,¹ Man-Sun Sy,¹ Shu G. Chen,¹ and Pierluigi Gambetti¹

Departments of ¹Pathology and ²Pharmacology, Case Western Reserve University, Cleveland, Ohio 44106, ³Department of Developmental and Molecular Biology, Albert Einstein College of Medicine, Bronx, New York 10461, ⁴National Veterinary Services Laboratories, United States Department of Agriculture, Ames, Iowa 50010, ⁵Animal Disease Research Unit, Agricultural Research Service, United States Department of Agriculture, Pullman, Washington 99164, and ⁶Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia 30333

Chronic wasting disease (CWD), a prion disease affecting free-ranging and captive cervids (deer and elk), is widespread in the United States and parts of Canada. The large cervid population, the popularity of venison consumption, and the apparent spread of the CWD epidemic are likely resulting in increased human exposure to CWD in the United States. Whether CWD is transmissible to humans, as has been shown for bovine spongiform encephalopathy (the prion disease of cattle), is unknown. We generated transgenic mice expressing the elk or human prion protein (PrP) in a PrP-null background. After intracerebral inoculation with elk CWD prion, two lines of "humanized" transgenic mice that are susceptible to human prions failed to develop the hallmarks of prion diseases after >657 and >756 d, respectively, whereas the "cervidized" transgenic mice became infected after 118–142 d. These data indicate that there is a substantial species barrier for transmission of elk CWD to humans.

Key words: chronic wasting disease; CWD; transmissibility to humans; transgenic mice; prion; cervids; deer; elk; species barrier

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Received June 16, 2005; revised July 18, 2005; accepted July 19, 2005.

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