Contributors: Wageningen BioVeterinary Research; Wageningen University and Research Wageningen (WUR); Istituto Superiore di Sanità = National Institute of Health (ISS); Istituto Zooprofilattico Sperimentale del Piemonte; IZSPLVA, Italian Reference Centre for Animal TSE; Infectiologie et Santé Publique (UMR ISP); Institut National de la Recherche Agronomique (INRA)-Université de Tours (UT); CEA- Saclay (CEA); Commissariat à l'énergie atomique et aux énergies alternatives (CEA); Médicaments et Technologies pour la Santé (MTS); Université Paris-Saclay-Institut des Sciences du Vivant Frédéric JOLIOT (JOLIOT); Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE); Service de Pharmacologie et Immunoanalyse (SPI); Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Université Paris-Saclay-Institut des Sciences du Vivant Frédéric JOLIOT (JOLIOT); Laboratoire d'Etudes et de Recherches en Immunoanalyses (LERI); Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Médicaments et Technologies pour la Santé (MTS); Interactions hôtes-agents pathogènes Toulouse (IHAP); Institut National de la Recherche Agronomique (INRA)-Ecole Nationale Vétérinaire de Toulouse (ENVT); Institut National Polytechnique (Toulouse) (Toulouse INP); Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National Polytechnique (Toulouse) (Toulouse INP); Université de Toulouse (UT)-Université de Toulouse (UT); Universidad de Zaragoza = University of Zaragoza Saragossa University = Université de Saragosse; Institute of Novel and Emerging Infectious Diseases (INNT); Friedrich-Loeffler-Institut (FLI); University of Edinburgh (Edin.); Royal Dick Sch Vet Studies, Roslin BioCtr, Roslin EH25 9PS; Animal and Plant Health Agency Addlestone, UK (APHA); Aristotle University of Thessaloniki; Hellenic Agricultural Organization Demeter (HAO Demeter); Veterinary Services of Cyprus; Ministry of Agriculture Rural Development and Environment; Partenaires INRAE; This work was supported by the European Commission: NeuroPrion EC FOOD‑CT‑2004‑506579 , GoatBSE EC FOOD‑CT‑2006‑36353 , GOAT‑TSE‑FREE ERA‑NET EMIDA and by National fundings: Netherlands—Ministry of Agriculture, Nature and Food Quality WOT 01‑002‑001.01; Italy—Italian Ministry of Health RF‑2009‑1474624; Spain—Ministry of Economy and Competitiveness AGL2012‑40071; United Kingdom—Department for Environment, Food and Rural Affairs SE2018 SE1961 , Biotechnology and Biological Sciences Research Council BB/J004332/1; Greece—Greek Ministry of Education; and France—Institut National de la Recherche Agronomique TRANSPRION EFA282/13 , REDPRION EFA148/16; European Project; European Project: 26570,NEUROPRION; European Project: 39519,GOATBSE; European Project: 36353,GOATBSE; European Project: 219235,EC:FP7:KBBE,FP7-ERANET-2007-RTD,EMIDA(2008)
نبذة مختصرة : International audience ; Scrapie in goats has been known since 1942, the archetype of prion diseases in which only prion protein (PrP) in misfolded state (PrPSc) 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 PrPSc (PrPres) 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 PrPresN-terminus. Suitable PrPres 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.
Processing Request
No Comments.