The emergence of classical BSE from atypical/Nor98 scrapie
Espinosa, Juan Carlos
Benestad, Sylvie L.
Thackray, Alana M.
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.
619 - Veterinària
National Academy of Sciences
Is part of
Proceedings of the National Academy of Sciences
Huor, Alvina, Juan Carlos Espinosa, Enric Vidal, Hervé Cassard, Jean-Yves Douet, Séverine Lugan, and Naima Aron et al. 2019. "The Emergence Of Classical BSE From Atypical/Nor98 Scrapie". Proceedings Of The National Academy Of Sciences 116 (52): 26853-26862. Proceedings of the National Academy of Sciences. doi:10.1073/pnas.1915737116.
Grant agreement number
EC/FP7/222887/EU/Protecting the food chain from prions: shaping European priorities through basic and applied research/PRIORITY
EC/INTERREG-POCTEFA/EU/EFA 282-13/ /TRANSPRION
MINECO/Programa Estatal de I+D+I orientada a los retos de la sociedad/AGL2016-78054-R/ES/ESTUDIO DEL IMPACTO DE LA SECUENCIA DE AMINOACIDOS DE PRP DEL HOSPEDADOR EN LA DIVERSIDAD Y ESTABILIDAD DE LAS CEPAS DE PRIONES/