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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...

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Autores principales: 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
Formato: Texto
Lenguaje:English
Publicado: Centers for Disease Control and Prevention 2007
Materias:
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.
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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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