title: Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France.
abstract: 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.
authors.affiliation: Institute for Novel and Emerging Infectious Diseases, Friedrich-Loeffler-Institut, Insel Riems, Germany. martin.groschup@fli.bund.de
authors.forename: Martin H
authors.initials: MH
authors.lastname: Groschup
authors.forename: Caroline
authors.initials: C
authors.lastname: Lacroux
authors.forename: Anne
authors.initials: A
authors.lastname: Buschmann
authors.forename: Gesine
authors.initials: G
authors.lastname: Lühken
authors.forename: Jacinthe
authors.initials: J
authors.lastname: Mathey
authors.forename: Martin
authors.initials: M
authors.lastname: Eiden
authors.forename: Séverine
authors.initials: S
authors.lastname: Lugan
authors.forename: Christine
authors.initials: C
authors.lastname: Hoffmann
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Espinosa
authors.forename: Thierry
authors.initials: T
authors.lastname: Baron
authors.forename: Juan Maria
authors.initials: JM
authors.lastname: Torres
authors.forename: Georg
authors.initials: G
authors.lastname: Erhardt
authors.forename: Olivier
authors.initials: O
authors.lastname: Andreoletti
mesh_terms: D000818:Animals; D001483:Base Sequence; D005260:Female; D005602:France; D020022:Genetic Predisposition to Disease; D005838:Genotype; D005858:Germany; D051379:Mice; D008822:Mice, Transgenic; D016133:Polymerase Chain Reaction / Q000379:methods; D018620:PrPSc Proteins / Q000235:genetics; D011328:Prions / Q000235:genetics* / Q000302:isolation & purification; D012608:Scrapie / Q000235:genetics*; D012756:Sheep / Q000235:genetics*
chemical_list: D018620:PrPSc Proteins; D011328:Prions