title: Transmission of scrapie prions to primate after an extended silent incubation period.
abstract: Classical bovine spongiform encephalopathy (c-BSE) is the only animal prion disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protective measures for animal and human health against animal prion diseases. Recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-BSE. We here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly relevant model for human prion diseases, after a 10-year silent incubation period, with features similar to those reported for human cases of sporadic CJD. Scrapie is thus actually transmissible to primates with incubation periods compatible with their life expectancy, although fourfold longer than BSE. Long-term experimental transmission studies are necessary to better assess the zoonotic potential of other prion diseases with high prevalence, notably Chronic Wasting Disease of deer and elk and atypical/Nor98 scrapie.
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Emmanuel E
authors.initials: EE
authors.lastname: Comoy
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Mikol
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Luccantoni-Freire
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Evelyne
authors.initials: E
authors.lastname: Correia
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Lescoutra-Etchegaray
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Durand
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Capucine
authors.initials: C
authors.lastname: Dehen
authors.affiliation: INRA-ENVT 23 Chemin des Capelles, 31076 Toulouse, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Andreoletti
authors.affiliation: Istituto Zooprofilattico Sperimentale del Piemonte, Via Bologna 148, 10154 Torino, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Casalone
authors.affiliation: Virus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, 1920 Dayton Avenue, 50010 Ames, Iowa, USA.
authors.forename: Juergen A
authors.initials: JA
authors.lastname: Richt
authors.affiliation: Virus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, 1920 Dayton Avenue, 50010 Ames, Iowa, USA.
authors.forename: Justin J
authors.initials: JJ
authors.lastname: Greenlee
authors.affiliation: ANSES, 59 Rue Tony Garnier, 69364 Lyon, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Baron
authors.affiliation: Norwegian Veterinary Institute, Ullevalvelen 68, 0106 Oslo, Norway.
authors.forename: Sylvie L
authors.initials: SL
authors.lastname: Benestad
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Paul
authors.initials: P
authors.lastname: Brown
authors.affiliation: Atomic Energy Commission (CEA), Institute of Emerging Diseases and Innovative therapies (iMETI), Division of Prions and related Diseases (SEPIA), 18 Route du Panorama, BP6, 92265 Fontenay-aux-Roses, France.
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Deslys
mesh_terms: D000818:Animals; D002417:Cattle; D002490:Central Nervous System / Q000378:metabolism / Q000473:pathology; D004195:Disease Models, Animal; D006801:Humans; D008252:Macaca fascicularis; D008297:Male; D051379:Mice; D011328:Prions / Q000378:metabolism; D012608:Scrapie / Q000473:pathology* / Q000635:transmission; D013702:Temporal Lobe / Q000473:pathology; D013997:Time Factors
chemical_list: D011328:Prions