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Human Prion Diseases and Neurodegeneration

  • Chapter
Prions Prions Prions

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 207))

Abstract

Investigations of neurodegeneration in humans and animals caused by prions have produced many unexpected and striking findings. For many years three uncommon human diseases and several animal disorders were labeled transmissible encephalopathies, spongiform encephalopathies, or slow virus diseases (GAJDUSEK 1977, 1985; SIGURDSSON 1954). These illnesses are transmissible to experimental animals after a prolonged incubation period, and some features of the transmissible pathogen resemble those of viruses. However, early attempts to characterize the infectious pathogen causing scrapie of sheep and goats argue that these transmissible agents differ from both viruses and viroids (ALPER et al. 1967; HUNTER 1972).

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Prusiner, S.B. (1996). Human Prion Diseases and Neurodegeneration. In: Prusiner, S.B. (eds) Prions Prions Prions. Current Topics in Microbiology and Immunology, vol 207. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-60983-1_1

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