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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 A(136)R(154)R(171) haplotype of the prion protein. This genotype was therefore assumed to confer resistance to BSE and classic scrapie under natural expo...
Autores principales: | , , , , , , , , , , , , |
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Formato: | Texto |
Lenguaje: | English |
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Centers for Disease Control and Prevention
2007
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2828083/ https://www.ncbi.nlm.nih.gov/pubmed/17953092 http://dx.doi.org/10.3201/eid1308.070077 |
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author | Groschup, Martin H. Lacroux, Caroline Buschmann, Anne Lühken, Gesine Mathey, Jacinthe Eiden, Martin Lugan, Séverine Hoffmann, Christine Espinosa, Juan Carlos Baron, Thierry Torres, Juan Maria Erhardt, Georg Andreoletti, Olivier |
author_facet | Groschup, Martin H. Lacroux, Caroline Buschmann, Anne Lühken, Gesine Mathey, Jacinthe Eiden, Martin Lugan, Séverine Hoffmann, Christine Espinosa, Juan Carlos Baron, Thierry Torres, Juan Maria Erhardt, Georg Andreoletti, Olivier |
author_sort | Groschup, Martin H. |
collection | PubMed |
description | In the past, natural scrapie and bovine spongiform encephalopathy (BSE) infections have essentially not been diagnosed in sheep homozygous for the A(136)R(154)R(171) 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. |
format | Text |
id | pubmed-2828083 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2007 |
publisher | Centers for Disease Control and Prevention |
record_format | MEDLINE/PubMed |
spelling | pubmed-28280832010-03-02 Classic Scrapie in Sheep with the ARR/ARR Prion Genotype in Germany and France Groschup, Martin H. Lacroux, Caroline Buschmann, Anne Lühken, Gesine Mathey, Jacinthe Eiden, Martin Lugan, Séverine Hoffmann, Christine Espinosa, Juan Carlos Baron, Thierry Torres, Juan Maria Erhardt, Georg Andreoletti, Olivier Emerg Infect Dis Research In the past, natural scrapie and bovine spongiform encephalopathy (BSE) infections have essentially not been diagnosed in sheep homozygous for the A(136)R(154)R(171) 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. Centers for Disease Control and Prevention 2007-08 /pmc/articles/PMC2828083/ /pubmed/17953092 http://dx.doi.org/10.3201/eid1308.070077 Text en https://creativecommons.org/licenses/by/4.0/This is a publication of the U.S. Government. This publication is in the public domain and is therefore without copyright. All text from this work may be reprinted freely. Use of these materials should be properly cited. |
spellingShingle | Research Groschup, Martin H. Lacroux, Caroline Buschmann, Anne Lühken, Gesine Mathey, Jacinthe Eiden, Martin Lugan, Séverine Hoffmann, Christine Espinosa, Juan Carlos Baron, Thierry Torres, Juan Maria Erhardt, Georg Andreoletti, Olivier Classic Scrapie in Sheep with the ARR/ARR Prion Genotype in Germany and France |
title | 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_short | 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 |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2828083/ https://www.ncbi.nlm.nih.gov/pubmed/17953092 http://dx.doi.org/10.3201/eid1308.070077 |
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