{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,6]],"date-time":"2025-08-06T13:53:08Z","timestamp":1754488388133,"version":"3.37.3"},"reference-count":48,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2024,2,24]],"date-time":"2024-02-24T00:00:00Z","timestamp":1708732800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/501100002915","name":"Fondation pour la Recherche M\u00e9dicale","doi-asserted-by":"publisher","award":["DEQ20150331689"],"award-info":[{"award-number":["DEQ20150331689"]}],"id":[{"id":"10.13039\/501100002915","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000277","name":"DEFRA","doi-asserted-by":"publisher","award":["SE1850","SE2019"],"award-info":[{"award-number":["SE1850","SE2019"]}],"id":[{"id":"10.13039\/501100000277","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007543","name":"Agence Nationale de S\u00e9curit\u00e9 du M\u00e9dicament et des Produits de Sant\u00e9","doi-asserted-by":"publisher","award":["2014S033 HAP ANSM 2014\/iPDB"],"award-info":[{"award-number":["2014S033 HAP ANSM 2014\/iPDB"]}],"id":[{"id":"10.13039\/501100007543","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008054","name":"Fundaci\u00f3n Ram\u00f3n Areces","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008054","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,25]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Atypical\/Nor98 scrapie (AS) is an idiopathic infectious prion disease affecting sheep and goats. Recent findings suggest that zoonotic prions from classical bovine spongiform encephalopathy (C-BSE) may copropagate with atypical\/Nor98 prions in AS sheep brains. Investigating the risk AS poses to humans is crucial.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>To assess the risk of sheep\/goat-to-human transmission of AS, we serially inoculated brain tissue from field and laboratory isolates into transgenic mice overexpressing human prion protein (Met129 allele). We studied clinical outcomes as well as presence of prions in brains and spleens.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>No transmission occurred on the primary passage, with no clinical disease or pathological prion protein in brains and spleens. On subsequent passages, 1 isolate gradually adapted, manifesting as prions with a phenotype resembling those causing MM1-type sporadic Creutzfeldt-Jakob disease in humans. However, further characterization using in vivo and in vitro techniques confirmed both prion agents as different strains, revealing a case of phenotypic convergence. Importantly, no C-BSE prions emerged in these mice, especially in the spleen, which is more permissive than the brain for C-BSE cross-species transmission.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>The results obtained suggest a low zoonotic potential for AS. Rare adaptation may allow the emergence of prions phenotypically resembling those spontaneously forming in humans.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/infdis\/jiae093","type":"journal-article","created":{"date-parts":[[2024,2,25]],"date-time":"2024-02-25T00:37:49Z","timestamp":1708821469000},"page":"161-171","source":"Crossref","is-referenced-by-count":3,"title":["Assessment of the Zoonotic Potential of Atypical Scrapie Prions in Humanized Mice Reveals Rare Phenotypic Convergence but Not Identity With Sporadic Creutzfeldt-Jakob Disease Prions"],"prefix":"10.1093","volume":"230","author":[{"given":"Alba","family":"Mar\u00edn-Moreno","sequence":"first","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Universit\u00e9 Versailles-Saint Quentin, Unit\u00e9 de Virologie Immunologie Mol\u00e9culaires , Jouy-en-Josas , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabienne","family":"Reine","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Universit\u00e9 Versailles-Saint Quentin, Unit\u00e9 de Virologie Immunologie Mol\u00e9culaires , Jouy-en-Josas , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laetitia","family":"Herzog","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Universit\u00e9 Versailles-Saint Quentin, Unit\u00e9 de Virologie Immunologie Mol\u00e9culaires , Jouy-en-Josas , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naima","family":"Aron","sequence":"additional","affiliation":[{"name":"Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Ecole Nationale V\u00e9t\u00e9rinaire de Toulouse, Unit\u00e9 Interactions H\u00f4te Agent Pathog\u00e8ne , Toulouse , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florence","family":"Jaffr\u00e9zic","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, AgroParisTech, Unit\u00e9 de G\u00e9n\u00e9tique Animale et Biologie Int\u00e9grative , Jouy-en-Josas , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Luc","family":"Vilotte","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, AgroParisTech, Unit\u00e9 de G\u00e9n\u00e9tique Animale et Biologie Int\u00e9grative , Jouy-en-Josas , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Human","family":"Rezaei","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Universit\u00e9 Versailles-Saint Quentin, Unit\u00e9 de Virologie Immunologie Mol\u00e9culaires , Jouy-en-Josas , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Andr\u00e9oletti","sequence":"additional","affiliation":[{"name":"Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Ecole Nationale V\u00e9t\u00e9rinaire de Toulouse, Unit\u00e9 Interactions H\u00f4te Agent Pathog\u00e8ne , Toulouse , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Davy","family":"Martin","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Universit\u00e9 Versailles-Saint Quentin, Unit\u00e9 de Virologie Immunologie Mol\u00e9culaires , Jouy-en-Josas , France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6706-5712","authenticated-orcid":false,"given":"Vincent","family":"B\u00e9ringue","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Universit\u00e9 Versailles-Saint Quentin, Unit\u00e9 de Virologie Immunologie Mol\u00e9culaires , Jouy-en-Josas , France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,2,24]]},"reference":[{"key":"2024072520124383100_jiae093-B1","doi-asserted-by":"crossref","first-page":"691","DOI":"10.3390\/ani11030691","article-title":"Classical and atypical scrapie in sheep and goats. 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