title: Unique properties of the classical bovine spongiform encephalopathy strain and its emergence from H-type bovine spongiform encephalopathy substantiated by VM transmission studies.
abstract: In addition to classical bovine spongiform encephalopathy (C-BSE), which is recognized as being at the origin of the human variant form of Creutzfeldt-Jakob disease, 2 rare phenotypes of BSE (H-type BSE [H-BSE] and L-type BSE [L-BSE]) were identified in 2004. H-type BSE and L-BSE are considered to be sporadic forms of prion disease in cattle because they differ from C-BSE with respect to incubation period, vacuolar pathology in the brain, and biochemical properties of the protease-resistant prion protein (PrP) in natural hosts and in some mouse models that have been tested. Recently, we showed that H-BSE transmitted to C57Bl/6 mice resulted in a dissociation of the phenotypic features, that is, some mice showed an H-BSE phenotype, whereas others had a C-BSE phenotype. Here, these 2 phenotypes were further studied in VM mice and compared with cattle C-BSE, H-BSE, and L-BSE. Serial passages from the C-BSE-like phenotype on VM mice retained similarities with C-BSE. Moreover, our results indicate that strains 301V and 301C derived from C-BSE transmitted to VM and C57Bl/6 mice, respectively, are fundamentally the same strain. These VM transmission studies confirm the unique properties of the C-BSE strain and support the emergence of a strain that resembles C-BSE from H-BSE.
authors: Unité Maladies Neurodégénératives, ANSES (Agence Nationale de Sécurité Sanitaire de l'Alimentation, de l'Environnement et du Travail) French Agency for Food, Environmental and Occupational Health Safety, Lyon, France. anna.bencsik@anses.fr
authors: Anna
authors: A
authors: Bencsik
authors: Mikael
authors: M
authors: Leboidre
authors: Sabine
authors: S
authors: Debeer
authors: Claire
authors: C
authors: Aufauvre
authors: Thierry
authors: T
authors: Baron
mesh_terms: D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000473:pathology* / Q000635:transmission*; D005260:Female; D051379:Mice; D008810:Mice, Inbred C57BL; D010641:Phenotype; D011328:Prions / Q000378:metabolism*; D012692:Serial Passage
chemical_list: D011328:Prions