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dc.contributor.authorThackray, Alana
dc.contributor.authorAndréoletti, Olivier
dc.contributor.authorBujdoso, Raymond
dc.date.accessioned2018-09-10T22:14:59Z
dc.date.available2018-09-10T22:14:59Z
dc.date.issued2018-09-01
dc.identifier.issn0006-8950
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/280007
dc.description.abstractMammalian prions propagate by template-directed misfolding and aggregation of normal cellular prion related protein PrPC as it converts into disease-associated conformers collectively referred to as PrPSc. Mammalian species may be permissive for prion disease because these hosts have co-evolved specific co-factors that assist PrPC conformational change and prion propagation. We have tested this hypothesis by examining whether faithful prion propagation occurs in the normally PrPC-null invertebrate host Drosophila melanogaster. Ovine PrP transgenic Drosophila exposed at the larval stage to ovine scrapie showed a progressive accumulation of transmissible prions in adult flies. Strikingly, the biological properties of distinct ovine prion strains were maintained during their propagation in Drosophila. Our observations show that the co-factors necessary for strain-specific prion propagation are not unique to mammalian species. Our studies establish Drosophila as a novel host for the study of transmissible mammalian prions.
dc.description.sponsorshipThis work was supported by funds from the NC3Rs Project (Grant NC/K000462/1).
dc.format.mediumPrint
dc.languageeng
dc.publisherOxford University Press (OUP)
dc.rightsAttribution 4.0 International
dc.rightsAttribution 4.0 International
dc.rightsAttribution 4.0 International
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.subjectAnimals
dc.subjectAnimals, Genetically Modified
dc.subjectMice, Transgenic
dc.subjectSheep
dc.subjectHumans
dc.subjectMice
dc.subjectDrosophila melanogaster
dc.subjectPrion Diseases
dc.subjectScrapie
dc.subjectDisease Models, Animal
dc.subjectDrosophila Proteins
dc.subjectPrions
dc.subjectPrion Proteins
dc.titleMammalian prion propagation in PrP transgenic Drosophila.
dc.typeArticle
prism.endingPage2710
prism.issueIdentifier9
prism.publicationDate2018
prism.publicationNameBrain
prism.startingPage2700
prism.volume141
dc.identifier.doi10.17863/CAM.23614
dcterms.dateAccepted2018-05-24
rioxxterms.versionofrecord10.1093/brain/awy183
rioxxterms.licenseref.urihttp://creativecommons.org/licenses/by/4.0/
rioxxterms.licenseref.startdate2018-09
dc.contributor.orcidThackray, Alana [0000-0002-2752-1127]
dc.contributor.orcidBujdoso, Raymond [0000-0002-5068-3247]
dc.identifier.eissn1460-2156
rioxxterms.typeJournal Article/Review
pubs.funder-project-idNational Centre for the Replacement Refinement and Reduction of Animals in Research (NC/K000462/1)
cam.issuedOnline2018-07-08
rioxxterms.freetoread.startdate2019-07-08


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Attribution 4.0 International
Except where otherwise noted, this item's licence is described as Attribution 4.0 International