Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France
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.
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dig-inia-es-20.500.12792-19932020-12-15T09:52:34Z Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France Groschup, M. H. Lacroux, C. Buschmann, A. Lühken, G. Mathey, J. Eiden, M. Lugan, S. Hoffmann, C. Espinosa, J. C. Baron, T. Torres, J. M. Erhardt, G. Andreoletti, O. 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. 2020-10-22T12:25:26Z 2020-10-22T12:25:26Z 2007 journal article http://hdl.handle.net/20.500.12792/1993 10.3201/eid1308.070077 eng Attribution-NonCommercial-ShareAlike 4.0 International http://creativecommons.org/licenses/by-nc-sa/4.0/ open access |
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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. |
format |
journal article |
author |
Groschup, M. H. Lacroux, C. Buschmann, A. Lühken, G. Mathey, J. Eiden, M. Lugan, S. Hoffmann, C. Espinosa, J. C. Baron, T. Torres, J. M. Erhardt, G. Andreoletti, O. |
spellingShingle |
Groschup, M. H. Lacroux, C. Buschmann, A. Lühken, G. Mathey, J. Eiden, M. Lugan, S. Hoffmann, C. Espinosa, J. C. Baron, T. Torres, J. M. Erhardt, G. Andreoletti, O. Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France |
author_facet |
Groschup, M. H. Lacroux, C. Buschmann, A. Lühken, G. Mathey, J. Eiden, M. Lugan, S. Hoffmann, C. Espinosa, J. C. Baron, T. Torres, J. M. Erhardt, G. Andreoletti, O. |
author_sort |
Groschup, M. H. |
title |
Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France |
title_short |
Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France |
title_full |
Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France |
title_fullStr |
Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France |
title_full_unstemmed |
Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France |
title_sort |
classic scrapie in sheep with the arr/arr prion genotype in germany and france |
publishDate |
2007 |
url |
http://hdl.handle.net/20.500.12792/1993 |
work_keys_str_mv |
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