title: Evidence for zoonotic potential of ovine scrapie prions.
abstract: Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
authors.affiliation: UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, 23 Chemin des Capelles, 31076 Toulouse, France.
authors.forename: Hervé
authors.initials: H
authors.lastname: Cassard
authors.affiliation: CISA- INIA, Valdeolmos, Madrid 28130, Spain.
authors.forename: Juan-Maria
authors.initials: JM
authors.lastname: Torres
authors.affiliation: UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, 23 Chemin des Capelles, 31076 Toulouse, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Lacroux
authors.affiliation: UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, 23 Chemin des Capelles, 31076 Toulouse, France.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Douet
authors.affiliation: Norwegian Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway.
authors.forename: Sylvie L
authors.initials: SL
authors.lastname: Benestad
authors.affiliation: INRA ISP, Centre INRA de Tours, 37380 Nouzilly, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Lantier
authors.affiliation: UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, 23 Chemin des Capelles, 31076 Toulouse, France.
authors.forename: Séverine
authors.initials: S
authors.lastname: Lugan
authors.affiliation: INRA ISP, Centre INRA de Tours, 37380 Nouzilly, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Lantier
authors.affiliation: UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, 23 Chemin des Capelles, 31076 Toulouse, France.
authors.forename: Pierrette
authors.initials: P
authors.lastname: Costes
authors.affiliation: UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, 23 Chemin des Capelles, 31076 Toulouse, France.
authors.forename: Naima
authors.initials: N
authors.lastname: Aron
authors.affiliation: UR892 Virologie et Immunologie Moléculaires Centre de Recherche de Jouy-en-Josas, F-78352 Jouy-en-Josas, France.
authors.forename: Fabienne
authors.initials: F
authors.lastname: Reine
authors.affiliation: UR892 Virologie et Immunologie Moléculaires Centre de Recherche de Jouy-en-Josas, F-78352 Jouy-en-Josas, France.
authors.forename: Laetitia
authors.initials: L
authors.lastname: Herzog
authors.affiliation: CISA- INIA, Valdeolmos, Madrid 28130, Spain.
authors.forename: Juan-Carlos
authors.initials: JC
authors.lastname: Espinosa
authors.affiliation: UR892 Virologie et Immunologie Moléculaires Centre de Recherche de Jouy-en-Josas, F-78352 Jouy-en-Josas, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Beringue
authors.affiliation: UMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, 23 Chemin des Capelles, 31076 Toulouse, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Andréoletti
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology / Q000635:transmission*; D016643:Encephalopathy, Bovine Spongiform / Q000473:pathology / Q000635:transmission*; D005260:Female; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D011328:Prions / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D012608:Scrapie / Q000473:pathology / Q000635:transmission*; D012756:Sheep; D015047:Zoonoses / Q000473:pathology / Q000635:transmission*
chemical_list: D011328:Prions