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• W1970744154 endingPage "310" @default.
• W1970744154 startingPage "297" @default.
• W1970744154 abstract "Scrapie and bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann-Sträussler-Scheinker diseases of humans are transmissible and genetic neurodegenerative diseases caused by prions. Infectious prion particles are composed largely, if not entirely, of an abnormal isoform of the prion protein which is encoded by a chromosomal gene. An as yet unidentified post-transla-tional process converts the cellular prion protein into an abnormal isoform. Scrapie neuropathology, incubation times, and prion synthesis in transgenic mice are controlled by the prion protein gene. Point mutations in the prion protein genes of animals and humans are genetically linked to development of neurodegeneration. Transgenic mice expressing mutant prion proteins spontaneously develop neurologic dysfunction and spongiform neuropathology. Studies of prion diseases may advance investigations of other neurodegenerative disorders and of how neurons differentiate, function for decades and grow senescent." @default.
• W1970744154 created "2016-06-24" @default.
• W1970744154 creator A5033786344 @default.
• W1970744154 creator A5066991343 @default.
• W1970744154 date "1991-07-01" @default.
• W1970744154 modified "2023-09-25" @default.
• W1970744154 title "Molecular Biology and Pathology of Scrapie and the Prion Diseases of Humans" @default.
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