Transmissible spongiform encephalopathy historical perspective
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Historical Perspective
- Natural Scrapie[1] is a infection that affects sheep and is caused by an unusual infectious agent.It was first described in 1732.
- Kuru is the first prion disease in humans which was described in 1957.It is a fatal exotic neurodegenerative disease that affects only people of a single language group in the remote mountainous interior of New Guinea.
- In 1959, veterinary pathologist W.J. Hadlow first recognized several similarities between Scrapie and Kuru.
- In 1959, I. Klatzo also noted that Kuru's histopathology resembled that of Creutzfeldt-Jakob disease (CJD), another fatal neurodegenerative progressive disease of unknown etiology that A.M. Jakob had first described in 1921.[2]
- Gajdusek, C.J. Gibbs, Jr., and M.P. Alpers used the existing knowledge of Scrapie and started efforts to transmit Kuru by inoculating Kuru brain tissue into non-human primates, that-although requiring several years-ultimately proved successful in 1965.Later Gajdusek and colleagues went on to demonstrate that not only the more common sporadic form of CJD but also familial CJD and a generally similar familial brain disease (Gerstmann-Sträussler-Scheinker syndrome) were also transmissible, first to non-human primates and later to other animals.[2]
- In 1982, [1]Stanley B. Prusiner formulated "prion hypothesis". Prusiner recognized that a misfolded form of a ubiquitous normal host protein was usually (if not always) detectable in tissues containing TSE agents, greatly facilitating the diagnosis and understanding their pathogenesis. Prusiner proposed that the TSE agent was likely to be composed partly (if not entirely )of the abnormal protein, for which he used the term "prion" protein and "prion" for the agent. Expression of the prion protein by animals-while not essential for life-was later found to be obligatory to infect them with TSEs
References
- ↑ 1.0 1.1 Liberski PP (2012). "Historical overview of prion diseases: a view from afar". Folia Neuropathol. 50 (1): 1–12. PMID 22505359.
- ↑ 2.0 2.1 Asher DM, Gregori L (2018). "Human transmissible spongiform encephalopathies: historic view". Handb Clin Neurol. 153: 1–17. doi:10.1016/B978-0-444-63945-5.00001-5. PMID 29887130.