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Publication : Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP.

First Author  Browning SR Year  2004
Journal  J Virol Volume  78
Issue  23 Pages  13345-50
PubMed ID  15542685 Mgi Jnum  J:94219
Mgi Id  MGI:3511628 Doi  10.1128/JVI.78.23.13345-13350.2004
Citation  Browning SR, et al. (2004) Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP. J Virol 78(23):13345-50
abstractText  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.
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