ArticleProceedings of the National Academy of Sciences of the United States of America2019
The emergence of classical BSE from atypical/Nor98 scrapie.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
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Who cites it
41 citing papers in PubMed, 67 citations in OpenAlex.
- Evidence for multiple prion conformers in natural scrapie isolates.PLoS pathogens · 2026Article
- Practical Real-Time Quaking-Induced Conversion for Detecting Classical Bovine Spongiform Encephalopathy and Classical and Atypical Scrapie Prions.Pathogens (Basel, Switzerland) · 2026Article
- Regulated coexpression of PrP from different species in mice impacts the replication and host range properties of prion strains.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- 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
- Article
- 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
- CRISPR/Cas9-editing of PRNP in Alpine goats.Veterinary research · 2025Article
- Zoonotic Potential of Chronic Wasting Disease after Adaptation in Intermediate Species.Emerging infectious diseases · 2024Article
- Convergent generation of atypical prions in knockin mouse models of genetic prion disease.The Journal of clinical investigation · 2024Article
- Characterisation of European Field Goat Prion Isolates in Ovine PrP Overexpressing Transgenic Mice (Tgshp IX) Reveals Distinct Prion Strains.Pathogens (Basel, Switzerland) · 2024Article
- BSE risk posed by ruminant collagen and gelatine derived from bones.EFSA journal. European Food Safety Authority · 2024Article
- Classical BSE dismissed as the cause of CWD in Norwegian red deer despite strain similarities between both prion agents.Veterinary research · 2024Article
- Diagnosis in Scrapie: Conventional Methods and New Biomarkers.Pathogens (Basel, Switzerland) · 2023Review
- Experimental transmission of ovine atypical scrapie to cattle.Veterinary research · 2023Article
- Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie.Veterinary research · 2023Article
- Prion strains: shining new light on old concepts.Cell and tissue research · 2023Review
- Development of a sensitive real-time quaking-induced conversion (RT-QuIC) assay for application in prion-infected blood.PloS one · 2023Article
- Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model.Acta neuropathologica communications · 2022Article
- Glycans are not necessary to maintain the pathobiological features of bovine spongiform encephalopathy.PLoS pathogens · 2022Article
- Article
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Authors and funding
19 authors at 7 institutions in 5 countries.
Funding
Abstract
Atypical/Nor98 scrapie (AS) is a prion disease of small ruminants. Currently there are no efficient measures to control this form of prion disease, and, importantly, the zoonotic potential and the risk that AS might represent for other farmed animal species remains largely unknown. In this study, we investigated the capacity of AS to propagate in bovine PrP transgenic mice. Unexpectedly, the transmission of AS isolates originating from 5 different European countries to bovine PrP mice resulted in the propagation of the classical BSE (c-BSE) agent. Detection of prion seeding activity in vitro by protein misfolding cyclic amplification (PMCA) demonstrated that low levels of the c-BSE agent were present in the original AS isolates. C-BSE prion seeding activity was also detected in brain tissue of ovine PrP mice inoculated with limiting dilutions (endpoint titration) of ovine AS isolates. These results are consistent with the emergence and replication of c-BSE prions during the in vivo propagation of AS isolates in the natural host. These data also indicate that c-BSE prions, a known zonotic agent in humans, can emerge as a dominant prion strain during passage of AS between different species. These findings provide an unprecedented insight into the evolution of mammalian prion strain properties triggered by intra- and interspecies passage. From a public health perspective, the presence of c-BSE in AS isolates suggest that cattle exposure to small ruminant tissues and products could lead to new occurrences of c-BSE.
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