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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Centers for Disease Control and Prevention (U.S.)
2007
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Online Access: | http://hdl.handle.net/20.500.12792/1993 http://hdl.handle.net/10261/293047 |
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dig-inia-es-10261-2930472023-02-20T07:35:16Z 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 Martín, Juan Carlos 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. 2023-02-20T07:35:16Z 2023-02-20T07:35:16Z 2007 artículo Emerging Infectious Diseases 13(8): 1201-1207 (2007) 1080-6040 http://hdl.handle.net/20.500.12792/1993 http://hdl.handle.net/10261/293047 10.3201/eid1308.070077 1080-6059 en open Centers for Disease Control and Prevention (U.S.) |
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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 |
artículo |
author |
Groschup, M. H. Lacroux, C. Buschmann, A. Lühken, G. Mathey, J. Eiden, M. Lugan, S. Hoffmann, C. Espinosa Martín, Juan Carlos 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 Martín, Juan Carlos 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 Martín, Juan Carlos 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 |
publisher |
Centers for Disease Control and Prevention (U.S.) |
publishDate |
2007 |
url |
http://hdl.handle.net/20.500.12792/1993 http://hdl.handle.net/10261/293047 |
work_keys_str_mv |
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