We report 2 natural scrapie cases in sheep carrying the ARR/ARR prion genotype, which is believed to confer resistance against classic scrapie and bovine spongiform encephalopathy

Andreoletti

Anne Buschmann

Baker

Baylis

Benestad

Bruce

Buschmann

Caroline Lacroux

Christine Hoffmann

Foster

Fraser

Georg Erhardt

Gesine Lühken

Goldmann

Houston

Ikeda

Ironside

Jacinthe Mathey

Juan Carlos Espinosa

Juan Maria Torres

Le Dur

Luhken

Martin Eiden

Martin H. Groschup

Moum

Olivier Andreoletti

Opinion of the Scientific Panel on Biological Hazards on

Ronzon

Séverine Lugan

Thierry Baron

Thuring

van Duijn

World Organization for Animal Health (OIE)

Emerging Infectious Diseases

English

PubMed

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 (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

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

ProdInra

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

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.

Digital.CSIC

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Crossref

Classic Scrapie in Sheep with the ARR/ARR Prion Genotype in Germany and France

Thierry G.M. Baron

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https://wwwnc.cdc.gov/eid/article/13/8/pdfs/07-0077.pdf

Classic Scrapie in Sheep with the ARR/ARR Prion Genotype in Germany and France

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