Skip to Main content Skip to Navigation
Journal articles

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

Abstract : 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.
Complete list of metadata

Cited literature [23 references]  Display  Hide  Download
Contributor : Valérie Cespedes Connect in order to contact the contributor
Submitted on : Wednesday, April 22, 2009 - 8:59:36 AM
Last modification on : Thursday, March 3, 2022 - 3:24:02 PM
Long-term archiving on: : Thursday, June 10, 2010 - 9:19:24 PM


Publisher files allowed on an open archive


  • HAL Id : hal-00377309, version 1
  • PRODINRA : 249775
  • PUBMED : 17953092
  • WOS : 000248530900011



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, Centers for Disease Control and Prevention, 2007, 13 (8), pp.1201-7. ⟨hal-00377309⟩



Record views


Files downloads