title: Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein.
abstract: The association between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) has demonstrated that cattle transmissible spongiform encephalopathies (TSEs) can pose a risk to human health and raises the possibility that other ruminant TSEs may be transmissible to humans. In recent years, several novel TSEs in sheep, cattle and deer have been described and the risk posed to humans by these agents is currently unknown. In this study, we inoculated two forms of atypical BSE (BASE and H-type BSE), a chronic wasting disease (CWD) isolate and seven isolates of atypical scrapie into gene-targeted transgenic (Tg) mice expressing the human prion protein (PrP). Upon challenge with these ruminant TSEs, gene-targeted Tg mice expressing human PrP did not show any signs of disease pathology. These data strongly suggest the presence of a substantial transmission barrier between these recently identified ruminant TSEs and humans.
authors.affiliation: Neuropathogenesis Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Roslin, Midlothian, UK.
authors.forename: Rona
authors.initials: R
authors.lastname: Wilson
authors.affiliation: Neuropathogenesis Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Roslin, Midlothian, UK.
authors.forename: Chris
authors.initials: C
authors.lastname: Plinston
authors.affiliation: Neuropathogenesis Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Roslin, Midlothian, UK.
authors.forename: Nora
authors.initials: N
authors.lastname: Hunter
authors.affiliation: Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Casalone
authors.affiliation: Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Turin, Italy.
authors.forename: Cristiano
authors.initials: C
authors.lastname: Corona
authors.affiliation: IRCCS Foundation, 'Carlo Besta' Neurological Institute, Milan, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Tagliavini
authors.affiliation: IRCCS Foundation, 'Carlo Besta' Neurological Institute, Milan, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Suardi
authors.affiliation: IRCCS Foundation, 'Carlo Besta' Neurological Institute, Milan, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Ruggerone
authors.affiliation: IRCCS Foundation, 'Carlo Besta' Neurological Institute, Milan, Italy.
authors.forename: Fabio
authors.initials: F
authors.lastname: Moda
authors.affiliation: Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Graziano
authors.affiliation: Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Sbriccoli
authors.affiliation: Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Cardone
authors.affiliation: Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Pocchiari
authors.affiliation: Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
authors.forename: Loredana
authors.initials: L
authors.lastname: Ingrosso
authors.affiliation: Agence Nationale de Sécurité Sanitaire, Lyon, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Baron
authors.affiliation: USDA, ARS, National Animal Disease Center, PO Box 70, Ames, IA 50010, USA.
authors.forename: Juergen
authors.initials: J
authors.lastname: Richt
authors.affiliation: UMR 1225 Interactions Hôtes-Agents Pathogènes, INRA, Ecole Nationale Vétérinaire, 23 chemin des Capelles, B.P. 87614, 31076 Toulouse Cedex 3, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Andreoletti
authors.affiliation: Neuropathology Section, Department of Pathology and Host Susceptibility, Animal Health and Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Marion
authors.initials: M
authors.lastname: Simmons
authors.affiliation: Neuropathology Section, Department of Pathology and Host Susceptibility, Animal Health and Veterinary Laboratories Agency, Addlestone, Surrey KT15 3NB, UK.
authors.forename: Richard
authors.initials: R
authors.lastname: Lockey
authors.affiliation: Neuropathogenesis Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Roslin, Midlothian, UK.
authors.forename: Jean C
authors.initials: JC
authors.lastname: Manson
authors.affiliation: Neuropathogenesis Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Roslin, Midlothian, UK.
authors.forename: Rona M
authors.initials: RM
authors.lastname: Barron
mesh_terms: D000818:Animals; D002417:Cattle; D003670:Deer; D004195:Disease Models, Animal; D016643:Encephalopathy, Bovine Spongiform / Q000635:transmission*; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D011328:Prions / Q000235:genetics / Q000502:physiology*; D018570:Risk Assessment; D012608:Scrapie / Q000635:transmission*; D012756:Sheep; D034081:Wasting Disease, Chronic / Q000635:transmission*; D015047:Zoonoses / Q000635:transmission
chemical_list: D011328:Prions