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dc.contributor.authorHuor, Alvinaen
dc.contributor.authorEspinosa, Juan Carlosen
dc.contributor.authorVidal, Enricen
dc.contributor.authorCassard, Hervéen
dc.contributor.authorDouet, Jean-Yvesen
dc.contributor.authorLugan, Séverineen
dc.contributor.authorAron, Naimaen
dc.contributor.authorMarín-Moreno, Albaen
dc.contributor.authorLorenzo, Patriciaen
dc.contributor.authorAguilar-Calvo, Patriciaen
dc.contributor.authorBadiola, Juanen
dc.contributor.authorBolea, Rosaen
dc.contributor.authorPumarola, Martíen
dc.contributor.authorBenestad, Sylvie Len
dc.contributor.authorOrge, Leonoreen
dc.contributor.authorThackray, Alanaen
dc.contributor.authorBujdoso, Raymonden
dc.contributor.authorTorres, Juan-Mariaen
dc.contributor.authorAndreoletti, Olivieren
dc.date.accessioned2019-11-26T00:30:55Z
dc.date.available2019-11-26T00:30:55Z
dc.date.issued2019-12-16en
dc.identifier.issn0027-8424
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/299238
dc.description.abstractAtypical/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 five 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 (end-point 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 zoonotic 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 inter-species 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.
dc.description.sponsorshipThis work was funded by FEDER POCTEFA TRANSPRION (EFA282/13) and REDPRION (EFA148/16), by the UK Food Standards Agency Exploring permeability of the species barrier (M03043 and FS231051), by the European Union through FP7 222887 “Priority”, the Spanish Ministerio de Economía y Competitividad [AGL2016-78054-R (AEI/FEDER, UE). A.M.-M. was supported by a fellowship from the INIA (FPI-SGIT-2015-02), and P.A.-C. was supported by a fellowship from the Spanish Ministerio de Economía y Competitividad (BES-2010-040922).
dc.format.mediumPrint-Electronicen
dc.languageengen
dc.publisherNational Academy of Sciences
dc.rightsAll rights reserved
dc.rights.uri
dc.titleThe emergence of classical BSE from atypical/Nor98 scrapie.en
dc.typeArticle
prism.publicationDate2019en
prism.publicationNameProceedings of the National Academy of Sciences of the United States of Americaen
dc.identifier.doi10.17863/CAM.46303
dcterms.dateAccepted2019-11-15en
rioxxterms.versionofrecord10.1073/pnas.1915737116en
rioxxterms.versionAM
rioxxterms.licenseref.urihttp://www.rioxx.net/licenses/all-rights-reserveden
rioxxterms.licenseref.startdate2019-12-16en
dc.contributor.orcidEspinosa, Juan Carlos [0000-0002-6719-9902]
dc.contributor.orcidVidal, Enric [0000-0002-4965-3286]
dc.contributor.orcidDouet, Jean-Yves [0000-0002-0426-4957]
dc.contributor.orcidMarín-Moreno, Alba [0000-0002-4023-6398]
dc.contributor.orcidPumarola, Martí [0000-0002-0935-7941]
dc.contributor.orcidOrge, Leonore [0000-0002-2673-2758]
dc.contributor.orcidThackray, Alana [0000-0002-2752-1127]
dc.contributor.orcidBujdoso, Raymond [0000-0002-5068-3247]
dc.contributor.orcidTorres, Juan-Maria [0000-0003-0443-9232]
dc.contributor.orcidAndreoletti, Olivier [0000-0002-7369-6016]
dc.identifier.eissn1091-6490
rioxxterms.typeJournal Article/Reviewen
pubs.funder-project-idAlberta Innovates - Health Solutions (via University of Calgary) (PEX17006-201700013)
pubs.funder-project-idNC3Rs (NC/R00093X/1)
cam.orpheus.successThu Jan 30 10:35:15 GMT 2020 - Embargo updated*
rioxxterms.freetoread.startdate2020-06-16


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