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Scrapie Prions and Degenerative Diseases

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Part of the book series: Neurologic Illness ((Ni))

Abstract

Almost two decades ago, a flurry of excitement surrounded studies on slow infectious agents. It was a time when an eight-year search for the cause of kuru was drawing to a close.1–3 Kuru had been classified as a degenerative neurologic disease of unknown origin that was confined to the Fore people and their neighboring tribes in the eastern highlands of Papua New Guinea. Almost every etiologic possibility for degenerative neurologic diseases had been considered with respect to kuru, but none had been identified.4 Based on pathologic and clinical similarities between kuru and scrapie, a neurologic disease of sheep, Hadlow suggested in 1959 that kuru might be caused by a slow infectious agent. Three years later, studies were begun by Gajdusek, Gibbs, and Alpers to test this hypothesis. In 1965, chimpanzees inoculated with brain tissue specimens from patients dying of kuru developed a kuru-like illness.2

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Prusiner, S.B. et al. (1984). Scrapie Prions and Degenerative Diseases. In: Thompson, R.A., Green, J.R. (eds) Infectious Diseases of the Central Nervous System. Neurologic Illness. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-6332-3_4

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  • DOI: https://doi.org/10.1007/978-94-011-6332-3_4

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