title: Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP.
abstract: 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 approximately 230 days following intracerebral inoculation with four CWD isolates. Inoculated transgenic mice homozygous for the transgene array developed disease after approximately 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.
authors.affiliation: Department of Microbiology, University of Kentucky, 800 Rose St., Lexington, KY 40536, USA.
authors.forename: Shawn R
authors.initials: SR
authors.lastname: Browning
authors.forename: Gary L
authors.initials: GL
authors.lastname: Mason
authors.forename: Tanya
authors.initials: T
authors.lastname: Seward
authors.forename: Mike
authors.initials: M
authors.lastname: Green
authors.forename: Gwyneth A J
authors.initials: GA
authors.lastname: Eliason
authors.forename: Candace
authors.initials: C
authors.lastname: Mathiason
authors.forename: Michael W
authors.initials: MW
authors.lastname: Miller
authors.forename: Elizabeth S
authors.initials: ES
authors.lastname: Williams
authors.forename: Ed
authors.initials: E
authors.lastname: Hoover
authors.forename: Glenn C
authors.initials: GC
authors.lastname: Telling
mesh_terms: D000818:Animals; D003670:Deer; D051379:Mice; D008822:Mice, Transgenic; D018620:PrPSc Proteins / Q000378:metabolism; D011328:Prions / Q000502:physiology*; D034081:Wasting Disease, Chronic / Q000209:etiology / Q000473:pathology / Q000635:transmission*
chemical_list: D018620:PrPSc Proteins; D011328:Prions