{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T06:32:58Z","timestamp":1781245978362,"version":"3.54.1"},"reference-count":49,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2018,3,1]],"date-time":"2018-03-01T00:00:00Z","timestamp":1519862400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2018,1,2]],"date-time":"2018-01-02T00:00:00Z","timestamp":1514851200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["U123160655"],"award-info":[{"award-number":["U123160655"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Department of Health's","award":["BRC 405\/HI\/JC\/104360"],"award-info":[{"award-number":["BRC 405\/HI\/JC\/104360"]}]}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","jns-journal.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of the Neurological Sciences"],"published-print":{"date-parts":[[2018,3]]},"DOI":"10.1016\/j.jns.2017.12.038","type":"journal-article","created":{"date-parts":[[2017,12,29]],"date-time":"2017-12-29T17:31:03Z","timestamp":1514568663000},"page":"4-11","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":9,"special_numbering":"C","title":["Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein"],"prefix":"10.1016","volume":"386","author":[{"given":"Susan","family":"Joiner","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuel A.","family":"Asante","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline M.","family":"Linehan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara","family":"Brock","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Brandner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan J.","family":"Bellworthy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marion M.","family":"Simmons","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Hope","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Collinge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4797-2259","authenticated-orcid":false,"given":"Jonathan D.F.","family":"Wadsworth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jns.2017.12.038_bb0005","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1038\/383685a0","article-title":"Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD","volume":"383","author":"Collinge","year":"1996","journal-title":"Nature"},{"key":"10.1016\/j.jns.2017.12.038_bb0010","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1038\/38925","article-title":"The same prion strain causes vCJD and BSE","volume":"389","author":"Hill","year":"1997","journal-title":"Nature"},{"key":"10.1016\/j.jns.2017.12.038_bb0015","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1038\/39057","article-title":"Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent","volume":"389","author":"Bruce","year":"1997","journal-title":"Nature"},{"key":"10.1016\/j.jns.2017.12.038_bb0020","doi-asserted-by":"crossref","first-page":"6358","DOI":"10.1093\/emboj\/cdf653","article-title":"BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein","volume":"21","author":"Asante","year":"2002","journal-title":"EMBO J."},{"key":"10.1016\/j.jns.2017.12.038_bb0025","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/S0140-6736(99)05128-4","article-title":"Variant Creutzfeldt-Jakob disease","volume":"354","author":"Collinge","year":"1999","journal-title":"Lancet"},{"key":"10.1016\/j.jns.2017.12.038_bb0030","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1016\/S0140-6736(06)68930-7","article-title":"Kuru in the 21st century\u2014an acquired human prion disease with very long incubation periods","volume":"367","author":"Collinge","year":"2006","journal-title":"Lancet"},{"key":"10.1016\/j.jns.2017.12.038_bb0035","doi-asserted-by":"crossref","first-page":"3725","DOI":"10.1098\/rstb.2008.0068","article-title":"A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea","volume":"363","author":"Collinge","year":"2008","journal-title":"Philos. Trans. R. Soc. Lond. Ser. B Biol. Sci."},{"key":"10.1016\/j.jns.2017.12.038_bb0040","doi-asserted-by":"crossref","first-page":"f5675","DOI":"10.1136\/bmj.f5675","article-title":"Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey","volume":"347","author":"Gill","year":"2013","journal-title":"BMJ"},{"key":"10.1016\/j.jns.2017.12.038_bb0045","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1126\/science.1218167","article-title":"Cell biology. The risk of prion zoonoses","volume":"335","author":"Collinge","year":"2012","journal-title":"Science"},{"key":"10.1016\/j.jns.2017.12.038_bb0050","doi-asserted-by":"crossref","first-page":"4197","DOI":"10.2903\/j.efsa.2015.4197","article-title":"Scientific opinion on a request for a review of a scientific publication concerning the zoonotic potential of ovine scrapie prions","volume":"13","author":"EFSA Panel on Biological Hazards (BIOHAZ Panel)","year":"2015","journal-title":"EFSA J."},{"key":"10.1016\/j.jns.2017.12.038_bb0055","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1111\/j.1365-2990.2010.01129.x","article-title":"Contribution of transgenic models to understanding human prion disease","volume":"36","author":"Wadsworth","year":"2010","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"10.1016\/j.jns.2017.12.038_bb0060","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1038\/352340a0","article-title":"Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease","volume":"352","author":"Palmer","year":"1991","journal-title":"Nature"},{"key":"10.1016\/j.jns.2017.12.038_bb0065","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1126\/science.1138718","article-title":"A general model of prion strains and their pathogenicity","volume":"318","author":"Collinge","year":"2007","journal-title":"Science"},{"key":"10.1016\/j.jns.2017.12.038_bb0070","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1038\/nature20415","article-title":"Mammalian prions and their wider relevance in neurodegenerative diseases","volume":"539","author":"Collinge","year":"2016","journal-title":"Nature"},{"key":"10.1016\/j.jns.2017.12.038_bb0075","doi-asserted-by":"crossref","first-page":"1793","DOI":"10.1126\/science.1103932","article-title":"Human prion protein with valine 129 prevents expression of variant CJD phenotype","volume":"306","author":"Wadsworth","year":"2004","journal-title":"Science"},{"key":"10.1016\/j.jns.2017.12.038_bb0080","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1146\/annurev.neuro.24.1.519","article-title":"Prion diseases of humans and animals: their causes and molecular basis","volume":"24","author":"Collinge","year":"2001","journal-title":"Annu. Rev. Neurosci."},{"key":"10.1016\/j.jns.2017.12.038_bb0085","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1051\/vetres:2008024","article-title":"Prion agents diversity and species barrier","volume":"39","author":"Beringue","year":"2008","journal-title":"Vet. Res."},{"key":"10.1016\/j.jns.2017.12.038_bb0090","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1007\/128_2011_166","article-title":"Transgenic mouse models and prion strains","volume":"305","author":"Telling","year":"2011","journal-title":"Top. Curr. Chem."},{"key":"10.1016\/j.jns.2017.12.038_bb0095","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/j.meegid.2014.06.010","article-title":"Infectious prion diseases in humans: cannibalism, iatrogenicity and zoonoses","volume":"26","author":"Haik","year":"2014","journal-title":"Infect. Genet. Evol."},{"key":"10.1016\/j.jns.2017.12.038_bb0100","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1093\/ilar\/ilv008","article-title":"The transmissible spongiform encephalopathies of livestock","volume":"56","author":"Greenlee","year":"2015","journal-title":"ILAR J."},{"key":"10.1016\/j.jns.2017.12.038_bb0105","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1136\/vr.156.16.523-b","article-title":"BSE agent signatures in a goat","volume":"156","author":"Eloit","year":"2005","journal-title":"Vet. Rec."},{"key":"10.1016\/j.jns.2017.12.038_bb0110","doi-asserted-by":"crossref","first-page":"2253","DOI":"10.3201\/eid1712.110333","article-title":"Isolation of prion with BSE properties from farmed goat","volume":"17","author":"Spiropoulos","year":"2011","journal-title":"Emerg. Infect. Dis."},{"key":"10.1016\/j.jns.2017.12.038_bb0115","doi-asserted-by":"crossref","first-page":"2099","DOI":"10.1099\/vir.0.81254-0","article-title":"Monitoring for bovine spongiform encephalopathy in sheep in great Britain, 1998-2004","volume":"87","author":"Stack","year":"2006","journal-title":"J. Gen. Virol."},{"key":"10.1016\/j.jns.2017.12.038_bb0120","doi-asserted-by":"crossref","first-page":"1875","DOI":"10.2903\/j.efsa.2010.1875","article-title":"Scientific opinion on BSE\/TSE infectivity in small ruminant tissues","volume":"8","author":"EFSA Panel on Biological Hazards (BIOHAZ)","year":"2010","journal-title":"EFSA J."},{"key":"10.1016\/j.jns.2017.12.038_bb0125","doi-asserted-by":"crossref","first-page":"1945","DOI":"10.2903\/j.efsa.2011.1945","article-title":"Joint scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans","volume":"9","author":"EFSA Panel on Biological Hazards (BIOHAZ)","year":"2011","journal-title":"EFSA J."},{"key":"10.1016\/j.jns.2017.12.038_bb0130","doi-asserted-by":"crossref","first-page":"1174","DOI":"10.1128\/JVI.01578-10","article-title":"Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep","volume":"85","author":"Plinston","year":"2010","journal-title":"J. Virol."},{"key":"10.1016\/j.jns.2017.12.038_bb0135","doi-asserted-by":"crossref","first-page":"e1001319","DOI":"10.1371\/journal.ppat.1001319","article-title":"Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice","volume":"7","author":"Padilla","year":"2011","journal-title":"PLoS Pathog."},{"key":"10.1016\/j.jns.2017.12.038_bb0140","doi-asserted-by":"crossref","first-page":"1731","DOI":"10.3201\/eid1911.121341","article-title":"Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein","volume":"19","author":"Wadsworth","year":"2013","journal-title":"Emerg. Infect. Dis."},{"key":"10.1016\/j.jns.2017.12.038_bb0145","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1038\/nature14510","article-title":"A naturally occurring variant of the human prion protein completely prevents prion disease","volume":"522","author":"Asante","year":"2015","journal-title":"Nature"},{"key":"10.1016\/j.jns.2017.12.038_bb0150","doi-asserted-by":"crossref","first-page":"e1004953","DOI":"10.1371\/journal.ppat.1004953","article-title":"Transmission properties of human PrP 102L prions challenge the relevance of mouse models of GSS","volume":"11","author":"Asante","year":"2015","journal-title":"PLoS Pathog."},{"key":"10.1016\/j.jns.2017.12.038_bb0155","doi-asserted-by":"crossref","first-page":"10759","DOI":"10.1073\/pnas.0604292103","article-title":"Dissociation of pathological and molecular phenotype of variant Creutzfeldt-Jakob disease in transgenic human prion protein 129 heterozygous mice","volume":"103","author":"Asante","year":"2006","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jns.2017.12.038_bb0160","doi-asserted-by":"crossref","first-page":"3885","DOI":"10.1073\/pnas.0800190105","article-title":"Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice","volume":"105","author":"Wadsworth","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jns.2017.12.038_bb0165","doi-asserted-by":"crossref","first-page":"764","DOI":"10.1099\/vir.0.005983-0","article-title":"Three serial passages of bovine spongiform encephalopathy in sheep do not significantly affect discriminatory test results","volume":"90","author":"Stack","year":"2009","journal-title":"J. Gen. Virol."},{"key":"10.1016\/j.jns.2017.12.038_bb0170","doi-asserted-by":"crossref","first-page":"1333","DOI":"10.1093\/brain\/awg125","article-title":"Molecular classification of sporadic Creutzfeldt-Jakob disease","volume":"126","author":"Hill","year":"2003","journal-title":"Brain"},{"key":"10.1016\/j.jns.2017.12.038_bb0175","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1016\/S0140-6736(96)91412-9","article-title":"A new variant of Creutzfeldt-Jakob disease in the UK","volume":"347","author":"Will","year":"1996","journal-title":"Lancet"},{"key":"10.1016\/j.jns.2017.12.038_bb0180","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1007\/978-1-59745-234-2_14","article-title":"Molecular diagnosis of human prion disease","volume":"459","author":"Wadsworth","year":"2008","journal-title":"Methods Mol. Biol."},{"key":"10.1016\/j.jns.2017.12.038_bb0185","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/S0140-6736(01)05403-4","article-title":"Tissue distribution of protease resistant prion protein in variant CJD using a highly sensitive immuno-blotting assay","volume":"358","author":"Wadsworth","year":"2001","journal-title":"Lancet"},{"key":"10.1016\/j.jns.2017.12.038_bb0190","doi-asserted-by":"crossref","first-page":"3688","DOI":"10.1128\/JVI.61.12.3688-3693.1987","article-title":"Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins","volume":"61","author":"Kascsak","year":"1987","journal-title":"J. Virol."},{"key":"10.1016\/j.jns.2017.12.038_bb0195","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1002\/path.2821","article-title":"Effect of fixation on brain and lymphoreticular vCJD prions and bioassay of key positive specimens from a retrospective vCJD prevalence study","volume":"223","author":"Wadsworth","year":"2011","journal-title":"J. Pathol."},{"key":"10.1016\/j.jns.2017.12.038_bb0200","doi-asserted-by":"crossref","first-page":"e1419","DOI":"10.1371\/journal.pone.0001419","article-title":"Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD","volume":"3","author":"Beringue","year":"2008","journal-title":"PLoS One"},{"key":"10.1016\/j.jns.2017.12.038_bb0205","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1002\/ana.410110406","article-title":"Measurement of the scrapie agent using an incubation time interval assay","volume":"11","author":"Prusiner","year":"1982","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.jns.2017.12.038_bb0210","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1136\/gut.2006.091637","article-title":"Prion infectivity in variant Creutzfeldt-Jakob disease rectum","volume":"56","author":"Wadsworth","year":"2007","journal-title":"Gut"},{"key":"10.1016\/j.jns.2017.12.038_bb0215","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1042\/BJ20081235","article-title":"Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin","volume":"416","author":"Cronier","year":"2008","journal-title":"Biochem. J."},{"key":"10.1016\/j.jns.2017.12.038_bb0220","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1099\/vir.0.026948-0","article-title":"Comparison of the level, distribution and form of disease-associated prion protein in variant and sporadic Creutzfeldt-Jakob disease brain using conformation dependent immunoassay and Western blot","volume":"92","author":"Choi","year":"2010","journal-title":"J. Gen. Virol."},{"key":"10.1016\/j.jns.2017.12.038_bb0225","doi-asserted-by":"crossref","first-page":"4347","DOI":"10.1038\/ncomms5347","article-title":"Prion neuropathology follows the accumulation of alternate prion protein isoforms after infective titre has peaked","volume":"5","author":"Sandberg","year":"2014","journal-title":"Nat. Commun."},{"key":"10.1016\/j.jns.2017.12.038_bb0230","doi-asserted-by":"crossref","first-page":"2518","DOI":"10.1099\/vir.0.045849-0","article-title":"Infectious titres of sheep scrapie and bovine spongiform encephalopathy agents cannot be accurately predicted from quantitative laboratory test results","volume":"93","author":"Gonzalez","year":"2012","journal-title":"J. Gen. Virol."},{"key":"10.1016\/j.jns.2017.12.038_bb0235","doi-asserted-by":"crossref","first-page":"e1001285","DOI":"10.1371\/journal.ppat.1001285","article-title":"Atypical\/Nor98 scrapie infectivity in sheep peripheral tissues","volume":"7","author":"Andreoletti","year":"2011","journal-title":"PLoS Pathog."},{"key":"10.1016\/j.jns.2017.12.038_bb0240","doi-asserted-by":"crossref","first-page":"848","DOI":"10.3201\/eid1705.101654","article-title":"Experimental oral transmission of atypical scrapie to sheep","volume":"17","author":"Simmons","year":"2011","journal-title":"Emerg. Infect. Dis."},{"key":"10.1016\/j.jns.2017.12.038_bb0245","doi-asserted-by":"crossref","first-page":"3747","DOI":"10.1098\/rstb.2008.0069","article-title":"Review. The origin of the prion agent of kuru: molecular and biological strain typing","volume":"363","author":"Wadsworth","year":"2008","journal-title":"Philos. Trans. R. Soc. Lond. Ser. B Biol. Sci."}],"container-title":["Journal of the Neurological Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022510X1734529X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022510X1734529X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2020,10,25]],"date-time":"2020-10-25T02:05:10Z","timestamp":1603591510000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0022510X1734529X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,3]]},"references-count":49,"alternative-id":["S0022510X1734529X"],"URL":"https:\/\/doi.org\/10.1016\/j.jns.2017.12.038","relation":{},"ISSN":["0022-510X"],"issn-type":[{"value":"0022-510X","type":"print"}],"subject":[],"published":{"date-parts":[[2018,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein","name":"articletitle","label":"Article Title"},{"value":"Journal of the Neurological Sciences","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jns.2017.12.038","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 The Authors. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}]}}