Abstract
Scrapie was the original member of what has become a family of both animal and human spongiform encephalopathies. Described clearly in the eighteenth century in both England and Germany as a fatal contagious disease of sheep, it was not experimentally transmitted until 1936, and became the subject of wide-ranging research in a number of laboratories in Great Britain. The human analog was first described in 1920 by the German neurologists Creutzfeldt and Jakob, and experimentally transmitted by Gajdusek in 1968, following a similar success in transmitting another analogous human disease (kuru) 2 years earlier. The evolving story of these and other members of the transmissible spongiform encephalopathy family (including “mad cow” disease) has led through a maze of studies involving many unexpected twists and turns, and eventually culminating in the discovery of a new category of infectious disease caused by the misfolding of a normal host protein (PrPTSE).
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Acknowledgments
The author apologizes to the many scientists whose names and contributions were omitted from this review due to the constraints of covering a very large subject in a very small space. He expresses his gratitude to Drs. O. Andeoletti, D.M. Asher, R. Bradley, K. Haynes, and R.H. Kimberlin for help in assuring factual accuracy, and especially to Drs. Bradley and Kimberlin for succumbing to the author’s plea for critical readings of the manuscript.
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Brown, P. (2013). Transmissible Spongiform Encephalopathy: From its Beginnings to Daniel Carlton Gajdusek. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5305-5_1
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