Chronic wasting disease of elk : Transmissibility to humans examined by transgenic mouse models

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

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214 Citations (Scopus)


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.
Original languageEnglish
Pages (from-to)7944 - 7949
Number of pages6
JournalThe Journal of Neuroscience
Issue number35
Publication statusPublished - 2005
Externally publishedYes

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