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Journal of Virology, December 2004, p. 13345-13350, Vol. 78, No. 23
0022-538X/04/$08.00+0     DOI: 10.1128/JVI.78.23.13345-13350.2004
Copyright © 2004, American Society for Microbiology. All Rights Reserved.

Transmission of Prions from Mule Deer and Elk with Chronic Wasting Disease to Transgenic Mice Expressing Cervid PrP

Shawn R. Browning,1 Gary L. Mason,2 Tanya Seward,3 Mike Green,4 Gwyneth A. J. Eliason,5 Candace Mathiason,5 Michael W. Miller,6 Elizabeth S. Williams,7 Ed Hoover,5 and Glenn C. Telling1,3,8*

Department of Microbiology, Immunology and Molecular Genetics,1 Sanders Brown Center on Aging,3 Department of Neurology,8 University of Kentucky Transgenic Facility, University of Kentucky, Lexington, Kentucky,4 Veterinary Diagnostic Laboratory,2 Department of Microbiology, Immunology and Pathology, Colorado State University,5 Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, Colorado,6 Department of Veterinary Sciences, University of Wyoming, Laramie, Wyoming7

Received 6 May 2004/ Accepted 3 August 2004

We generated mice expressing cervid prion protein to produce a transgenic system simulating chronic wasting disease (CWD) in deer and elk. While normal mice were resistant to CWD, these transgenic mice uniformly developed signs of neurological dysfunction ~230 days following intracerebral inoculation with four CWD isolates. Inoculated transgenic mice homozygous for the transgene array developed disease after ~160 days. The brains of sick transgenic mice exhibited widespread spongiform degeneration and contained abnormal prion protein and abundant amyloid plaques, many of which were florid plaques. Transmission studies indicated that the same prion strain caused CWD in the analyzed mule deer and elk. These mice provide a new and reliable tool for detecting CWD prions.

* Corresponding author. Mailing address: 332 Health Sciences Research Building, University of Kentucky, 800 Rose St., Lexington, KY 40536. Phone: (859) 323-8564. Fax: (859) 257-6151. E-mail: gtell2{at}uky.edu.

Journal of Virology, December 2004, p. 13345-13350, Vol. 78, No. 23
0022-538X/04/$08.00+0     DOI: 10.1128/JVI.78.23.13345-13350.2004
Copyright © 2004, American Society for Microbiology. All Rights Reserved.



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