title: Isolation from cattle of a prion strain distinct from that causing bovine spongiform encephalopathy.
abstract: To date, bovine spongiform encephalopathy (BSE) and its human counterpart, variant Creutzfeldt-Jakob disease, have been associated with a single prion strain. This strain is characterised by a unique and remarkably stable biochemical profile of abnormal protease-resistant prion protein (PrP(res)) isolated from brains of affected animals or humans. However, alternate PrP(res) signatures in cattle have recently been discovered through large-scale screening. To test whether these also represent separate prion strains, we inoculated French cattle isolates characterised by a PrP(res) of higher apparent molecular mass--called H-type--into transgenic mice expressing bovine or ovine PrP. All mice developed neurological symptoms and succumbed to these isolates, showing that these represent a novel strain of infectious prions. Importantly, this agent exhibited strain-specific features clearly distinct from that of BSE agent inoculated to the same mice, which were retained on further passage. Moreover, it also differed from all sheep scrapie isolates passaged so far in ovine PrP-expressing mice. Our findings therefore raise the possibility that either various prion strains may exist in cattle, or that the BSE agent has undergone divergent evolution in some animals.
authors.affiliation: Institut National de la Recherche Agronomique, Virologie Immunologie Moléculaires, Jouy-en-Josas, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Béringue
authors.forename: Anna
authors.initials: A
authors.lastname: Bencsik
authors.forename: Annick
authors.initials: A
authors.lastname: Le Dur
authors.forename: Fabienne
authors.initials: F
authors.lastname: Reine
authors.forename: Thanh Lan
authors.initials: TL
authors.lastname: Laï
authors.forename: Nathalie
authors.initials: N
authors.lastname: Chenais
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Tilly
authors.forename: Anne-Gaëlle
authors.initials: AG
authors.lastname: Biacabé
authors.forename: Thierry
authors.initials: T
authors.lastname: Baron
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Vilotte
authors.forename: Hubert
authors.initials: H
authors.lastname: Laude
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D002417:Cattle; D004198:Disease Susceptibility; D016643:Encephalopathy, Bovine Spongiform / Q000378:metabolism* / Q000473:pathology / Q000635:transmission; D008136:Longevity; D051379:Mice; D008822:Mice, Transgenic; D018620:PrPSc Proteins / Q000302:isolation & purification* / Q000378:metabolism*; D012756:Sheep; D013045:Species Specificity
chemical_list: D018620:PrPSc Proteins