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Cold Spring Harb Perspect Med. 2017 Nov 1;7(11). pii: a027151. doi: 10.1101/cshperspect.a027151.

Experimental Models of Inherited PrP Prion Diseases.

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Tanz Centre for Research in Neurodegenerative Diseases and Department of Biochemistry, University of Toronto, Toronto, Ontario M5T 2S8, Canada.
Institute for Neurodegenerative Diseases, Departments of Neurology and Biochemistry and Biophysics, Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, California 94143.


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.

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