Journal
JOURNAL OF VIROLOGY
Volume 78, Issue 23, Pages 13345-13350Publisher
AMER SOC MICROBIOLOGY
DOI: 10.1128/JVI.78.23.13345-13350.2004
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Funding
- NIAID NIH HHS [N01AI25491] Funding Source: Medline
- NINDS NIH HHS [R01 NS/AI0334] Funding Source: Medline
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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 similar to230 days following intracerebral inoculation with four CWD isolates. Inoculated transgenic mice homozygous for the transgene array developed disease after similar to160 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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