title: Experimental transmission of two young and one suspended bovine spongiform encephalopathy (BSE) cases to bovinized transgenic mice.
abstract: Bovine spongiform encephalopathy (BSE) is caused by a prion that primarily consists of an abnormal isoform of the prion protein (PrP(Sc)). Since PrP(Sc) is partially resistant to proteolytic digestion, the routine diagnosis of BSE is based on the immunological detection of the proteinase K (PK)-resistant moiety of PrP(Sc) (PrP(core)). However, transmission studies are indispensable in order to demonstrate prion infectivity and to analyze prion characteristics. Transmission experiments were accordingly performed on 2 young BSE cases (BSE/JP8, BSE/JP9) and 1 suspected BSE case (Suspended-1) that were detected by the BSE screening program in Japan. In this study, we attempted to transmit the prion from these 3 animals by using transgenic mice overexpressing bovine PrP (TgBoPrP). In spite of the use of BSE-sensitive transgenic mice, none of the mice developed neurological signs nor accumulated PrP(Sc) in their brains for more than 600 days post-inoculation, even with subsequent blind passages. The results of a dilution experiment using the classical BSE prion indicated that prion infectivity in these 3 cattle was below the detection limit of 10(3.0) LD(50)/g.
authors: Prion Disease Research Center, National Institute of Animal Health, Ibaraki 305-0856, Japan. tyoko@affrc.go.jp
authors: Takashi
authors: T
authors: Yokoyama
authors: Kentaro
authors: K
authors: Masujin
authors: Yoshio
authors: Y
authors: Yamakawa
authors: Tetsutaro
authors: T
authors: Sata
authors: Yuichi
authors: Y
authors: Murayama
authors: Yujing
authors: Y
authors: Shu
authors: Hiroyuki
authors: H
authors: Okada
authors: Shirou
authors: S
authors: Mohri
authors: Morikazu
authors: M
authors: Shinagawa
mesh_terms: D000818:Animals; D015153:Blotting, Western; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000175:diagnosis / Q000378:metabolism / Q000635:transmission*; D019286:Endopeptidase K / Q000378:metabolism; D007150:Immunohistochemistry; D051379:Mice; D008822:Mice, Transgenic; D023421:Models, Animal; D018621:PrPC Proteins / Q000378:metabolism* / Q000472:pathogenicity; D018620:PrPSc Proteins / Q000378:metabolism* / Q000472:pathogenicity
chemical_list: D018621:PrPC Proteins; D018620:PrPSc Proteins; D019286:Endopeptidase K