TY - JOUR AU - Groschup, Martin H. AU - Lacroux, Caroline AU - Buschmann, Anne AU - Lühken, Gesine AU - Mathey, Jacinthe AU - Eiden, Martin AU - Lugan, Séverine AU - Hoffmann, Christine AU - Espinosa, Juan Carlos AU - Baron, Thierry G.M. AU - Torres, Juan Maria AU - Erhardt, Georg AU - Andreoletti, Olivier T1 - Classic Scrapie in Sheep with the ARR/ARR Prion Genotype in Germany and France T2 - Emerging Infectious Disease journal PY - 2007 VL - 13 IS - 8 SP - 1201 SN - 1080-6059 AB - 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 (PrPSc) was associated with a lower proteinase-K resistance. PrPSc 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. KW - Prion KW - scrapie KW - bovine spongiform encephalopathy KW - research KW - France KW - Germany DO - 10.3201/eid1308.070077 UR - https://wwwnc.cdc.gov/eid/article/13/8/07-0077_article ER - End of Reference