Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods - INRAE - Institut national de recherche pour l’agriculture, l’alimentation et l’environnement Access content directly
Journal Articles Veterinary Research Year : 2019

Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods

Jan Langeveld
Laura Pirisinu
  • Function : Author
Jorg Jacobs
  • Function : Author
Maria Mazza
  • Function : Author
Isabelle Lantier
  • Function : Author
Olivier Andréoletti
  • Function : Author
Cristina Acin
  • Function : Author
Elena Esposito
  • Function : Author
Christine Fast
  • Function : Author
Martin Groschup
  • Function : Author
Wilfred Goldmann
  • Function : Author
John Spiropoulos
  • Function : Author
Theodoros Sklaviadis
  • Function : Author
Frederic Lantier
  • Function : Author
Loukia Ekateriniadou
  • Function : Author
Penelope Papasavva-Stylianou
  • Function : Author
Lucien van Keulen
  • Function : Author
Pier-Luigi Acutis
  • Function : Author
Umberto Agrimi
  • Function : Author
Alex Bossers
  • Function : Author
Romolo Nonno
  • Function : Author

Abstract

Abstract Scrapie in goats has been known since 1942, the archetype of prion diseases in which only prion protein (PrP) in misfolded state (PrP Sc ) acts as infectious agent with fatal consequence. Emergence of bovine spongiform encephalopathy (BSE) with its zoonotic behaviour and detection in goats enhanced fears that its source was located in small ruminants. However, in goats knowledge on prion strain typing is limited. A European-wide study is presented concerning the biochemical phenotypes of the protease resistant fraction of PrP Sc (PrP res ) in over thirty brain isolates from transmissible spongiform encephalopathy (TSE) affected goats collected in seven countries. Three different scrapie forms were found: classical scrapie (CS), Nor98/atypical scrapie and one case of CH1641 scrapie. In addition, CS was found in two variants—CS-1 and CS-2 (mainly Italy)—which differed in proteolytic resistance of the PrP res N - terminus . Suitable PrP res markers for discriminating CH1641 from BSE (C-type) appeared to be glycoprofile pattern, presence of two triplets instead of one, and structural (in)stability of its core amino acid region. None of the samples exhibited BSE like features. BSE and these four scrapie types, of which CS-2 is new, can be recognized in goats with combinations of a set of nine biochemical parameters.

Dates and versions

hal-04449965 , version 1 (09-02-2024)

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Cite

Jan Langeveld, Laura Pirisinu, Jorg Jacobs, Maria Mazza, Isabelle Lantier, et al.. Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods. Veterinary Research, 2019, 50 (1), pp.97. ⟨10.1186/s13567-019-0718-z⟩. ⟨hal-04449965⟩
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