Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France.

Martin H Groschup (1) , Caroline Lacroux (2) , Anne Buschmann (1) , Gesine Lühken (3) , Jacinthe Mathey (2) , Martin Eiden (1) , Séverine Lugan (2) , Christine Hoffmann (1) , Juan Carlos Espinosa (4) , Thierry Baron (5) , Juan Maria Torres (4) , Georg Erhardt (3) , Olivier Andreoletti (2)

1 FLI - Friedrich-Loeffler-Institut
2 INRA - Institut National de la Recherche Agronomique
3 JLU - Justus-Liebig-Universität Gießen = Justus Liebig University
4 INIA-CISA - Centro de Investigacion en Sanidad Animal
5 Laboratoire d'études et de recherches en pathologie bovine et hygiène des viandes

In the past, natural scrapie and bovine spongiform encephalopathy (BSE) infections have essentially not been diagnosed in sheep homozygous for the A136R154R171 haplotype of the prion protein. This genotype was therefore assumed to confer resistance to BSE and classic scrapie under natural exposure conditions. Hence, to exclude prions from the human food chain, massive breeding efforts have been undertaken in the European Union to amplify this gene. We report the identification of 2 natural scrapie cases in ARR/ARR sheep that have biochemical and transmission characteristics similar to cases of classic scrapie, although the abnormally folded prion protein (PrP(Sc)) was associated with a lower proteinase-K resistance. PrP(Sc) was clearly distinct from BSE prions passaged in sheep and from atypical scrapie prions. These findings strongly support the idea that scrapie prions are a mosaic of agents, which harbor different biologic properties, rather than a unique entity.

Keywords: XUATNC; HAFSSA; ESST

Domains: Veterinary medicine and animal Health; Animal genetics

Martin H Groschup, Caroline Lacroux, Anne Buschmann, Gesine Lühken, Jacinthe Mathey, et al.. Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France.. Emerging Infectious Diseases, 2007, 13 (8), pp.1201-7. ⟨hal-00377309⟩