ArticlePloS one2011
Atypical scrapie isolates involve a uniform prion species with a complex molecular signature.
Article in PloS one, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 24 citations in OpenAlex.
- Co-presence of classical scrapie but not classical Bovine Spongiform Encephalopathy in transmissions from Dutch sheep with atypical scrapie.The Journal of general virology · 2025Article
- Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- A single amino acid residue in bank vole prion protein drives permissiveness to Nor98/atypical scrapie and the emergence of multiple strain variants.PLoS pathogens · 2022Article
- Circulation of Nor98 Atypical Scrapie in Portuguese Sheep Confirmed by Transmission of Isolates into Transgenic Ovine ARQ-PrP Mice.International journal of molecular sciences · 2021Article
- Association of chronic wasting disease susceptibility with prion protein variation in white-tailed deer (Prion · 2020Article
- The Structure of PrPPathogens (Basel, Switzerland) · 2018Review
- Transmission of atypical scrapie to homozygous ARQ sheep.The Journal of veterinary medical science · 2016Article
- Prions: Beyond a Single Protein.Clinical microbiology reviews · 2016Review
- Deer Prion Proteins Modulate the Emergence and Adaptation of Chronic Wasting Disease Strains.Journal of virology · 2015Article
- Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections.The Journal of general virology · 2015Article
- Bank Vole Prion Protein As an Apparently Universal Substrate for RT-QuIC-Based Detection and Discrimination of Prion Strains.PLoS pathogens · 2015Article
- Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein.Emerging infectious diseases · 2013Article
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- Prion protein misfolding, strains, and neurotoxicity: an update from studies on Mammalian prions.International journal of cell biology · 2013Review
- Article
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The pathobiology of atypical scrapie, a prion disease affecting sheep and goats, is still poorly understood. In a previous study, we demonstrated that atypical scrapie affecting small ruminants in Switzerland differs in the neuroanatomical distribution of the pathological prion protein (PrP(d)). To investigate whether these differences depend on host-related vs. pathogen-related factors, we transmitted atypical scrapie to transgenic mice over-expressing the ovine prion protein (tg338). The clinical, neuropathological, and molecular phenotype of tg338 mice is similar between mice carrying the Swiss atypical scrapie isolates and the Nor98, an atypical scrapie isolate from Norway. Together with published data, our results suggest that atypical scrapie is caused by a uniform type of prion, and that the observed phenotypic differences in small ruminants are likely host-dependant. Strikingly, by using a refined SDS-PAGE technique, we established that the prominent proteinase K-resistant prion protein fragment in atypical scrapie consists of two separate, unglycosylated peptides with molecular masses of roughly 5 and 8 kDa. These findings show similarities to those for other prion diseases in animals and humans, and lay the groundwork for future comparative research.
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