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Volume 26, Number 6—June 2020
Etymologia

Etymologia: Scrapie

Ronnie HenryComments to Author  and Lawrence B. Schonburger
Author affiliations: Centers for Disease Control and Prevention, Atlanta, Georgia, USA

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Scrapie [skraʹpe]

Figure

Thumbnail of This photomicrograph of a neural tissue specimen, harvested from a scrapie affected mouse, revealed the presence of prion protein stained in red, which was in the process of being trafficked between neurons, by way of their interneuronal connections, known as neurites. Prion proteins can become infectious, causing neurodegenerative diseases such as transmissible spongiform encephalopathies (TSEs), which includes bovine spongiform encephalopathy (BSE), more commonly referred to a mad

Figure. This photomicrograph of a neural tissue specimen, harvested from a scrapie affected mouse, revealed the presence of prion protein stained in red, which was in the process of being trafficked between...

Scrapie is a fatal neurodegenerative disease of sheep and goats that was the first of a group of spongiform encephalopathies to be reported (1732 in England) and the first whose transmissibility was demonstrated by Cuille and Chelle in 1936 (Figure). The name resulted because most affected sheep develop pruritis and compulsively scratch their hides against fixed objects. Like other transmissible spongiform encephalopathies (TSEs), scrapie is associated with an alteration in conformation of a normal neural cell glycoprotein, the prion protein (PrPC). The scrapie agent was first described as a prion (and the term coined) by Stanley Prusiner in 1982, work for which he received the Nobel Prize in 1997.

The altered, misfolded form, designated PrP scrapie (PrPSc), aggregates and is thought to be an essential component of the infectious particle that causes TSEs. PrPSc is often used to designate the infectious particle responsible for all TSEs, including those in humans, such as Creutzfeldt-Jakob disease, even though scrapie does not appear to affect humans.

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References

  1. Brown  P, Bradley  R. 1755 and all that: a historical primer of transmissible spongiform encephalopathy. BMJ. 1998;317:168892. DOIPubMed
  2. Cuillé  J, Chelle  PL. The so-called “trembling” disease of sheep: is it inoculable? [in French]. Comptes Rendus de l’Académie Sciences. 1936;203:1552.
  3. Laplanche  J-L, Hunter  N, Shinagawa  M, Williams  E. Scrapie, chronic wasting disease, and transmissible mink encephalopathy. In: Prusiner SB, editor. Prion biology and diseases. Cold Spring Harbor (NY): Cold Spring Harbor Laboratory Press; 1999. p. 393–429.
  4. Prusiner  SB. Novel proteinaceous infectious particles cause scrapie. Science. 1982;216:13644. DOIPubMed

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Cite This Article

DOI: 10.3201/eid2606.et2606

Original Publication Date: May 04, 2020

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Table of Contents – Volume 26, Number 6—June 2020

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Ronnie Henry, Centers for Disease Control and Prevention, 1600 Clifton Rd NE, Mailstop V18-2, Atlanta, GA 30329-4027, USA

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Page created: May 18, 2020
Page updated: May 18, 2020
Page reviewed: May 18, 2020
The conclusions, findings, and opinions expressed by authors contributing to this journal do not necessarily reflect the official position of the U.S. Department of Health and Human Services, the Public Health Service, the Centers for Disease Control and Prevention, or the authors' affiliated institutions. Use of trade names is for identification only and does not imply endorsement by any of the groups named above.
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