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The biology of prions and the diseases wrought by these agents have become a fascinating area of investigation. Prions produce progressive neurologic disease in multiple species that include scrapie, bovine spongiform encephalopathy (BSE; "mad cow disease"), kuru, Gerstmann-Sträussler syndrome, fatal familial insomnia, and Creutzfeldt-Jakob disease. The prevailing and increasingly substantiated view is that disease is produced by unconventional proteins, PrPc, encoded by the mammalian genome that become pathogenic when they adopt an altered conformation either spontaneously owing to mutation or through an induced alteration secondary to inoculation with pathogenic forms of the protein (PrPsc). Elucidation of the mechanisms underlying pathogenic PrP folding will define a new paradigm for inherited and acquired neurodegenerative disease. The approaches used by investigators to examine these agents, their diseases, and aspects of their impact are the diverse charge of Prion Diseases, a volume in the Methods in Molecular Medicine series.
Prion Diseases is
Federoff HJ. Prion Diseases. Arch Neurol. 1997;54(3):231. doi:10.1001/archneur.1997.00550150007004