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- W2575675571 endingPage "a027151" @default.
- W2575675571 startingPage "a027151" @default.
- W2575675571 abstract "The inherited prion protein (PrP) prion disorders, which include familial Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia, constitute ∼10%-15% of all PrP prion disease cases in humans. Attempts to generate animal models of these disorders using transgenic mice expressing mutant PrP have produced variable results. Although many lines of mice develop spontaneous signs of neurological illness with accompanying prion disease-specific neuropathological changes, others do not. Furthermore, demonstrating the presence of protease-resistant PrP species and prion infectivity-two of the hallmarks of the PrP prion disorders-in the brains of spontaneously sick mice has proven particularly challenging. Here, we review the progress that has been made toward developing accurate mouse models of the inherited PrP prion disorders." @default.
- W2575675571 created "2017-01-26" @default.
- W2575675571 creator A5009077284 @default.
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- W2575675571 date "2017-01-17" @default.
- W2575675571 modified "2023-10-16" @default.
- W2575675571 title "Experimental Models of Inherited PrP Prion Diseases" @default.
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- W2575675571 doi "https://doi.org/10.1101/cshperspect.a027151" @default.
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