title: Atypical BSE in Germany--proof of transmissibility and biochemical characterization.
abstract: Intensive active surveillance has uncovered two atypical German BSE cases in older cattle which resemble the two different atypical BSE phenotypes that have recently been described in France (designated H-type) and Italy (designated L-type or BASE). The H-type is characterized by a significantly higher molecular size, but a conventional glycopattern of the proteinase K treated abnormal prion protein (PrP(Sc)), while the L-type PrP(Sc) has only a slightly lower molecular size and a distinctly different glycopattern. In this paper we describe the successful transmission of both German atypical BSE cases to transgenic mice overexpressing bovine PrP(C). Upon challenge with the L-type, these mice developed BSE after a substantially shorter incubation period than any classical BSE transmission using these mice to date. In contrast, the incubation period was distinctly prolonged when these mice were challenged with the H-type. PrP(Sc) accumulated in the brains of these mice were of the same atypical BSE type that had been used for the transmission. These atypical cases suggest the possible existence of sporadic BSE cases in bovines. It is thus feasible that the BSE epidemic in the UK could have also been initiated by an intraspecies transmission from a sporadic BSE case.
authors.affiliation: Friedrich-Loeffler-Institut (FLI), Institute for Novel and Emerging Infectious Diseases, Boddenblick 5a, 17493 Greifswald, Insel Riems, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Buschmann
authors.forename: A
authors.initials: A
authors.lastname: Gretzschel
authors.forename: A-G
authors.initials: AG
authors.lastname: Biacabe
authors.forename: K
authors.initials: K
authors.lastname: Schiebel
authors.forename: C
authors.initials: C
authors.lastname: Corona
authors.forename: C
authors.initials: C
authors.lastname: Hoffmann
authors.forename: M
authors.initials: M
authors.lastname: Eiden
authors.forename: T
authors.initials: T
authors.lastname: Baron
authors.forename: C
authors.initials: C
authors.lastname: Casalone
authors.forename: Martin H
authors.initials: MH
authors.lastname: Groschup
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001921:Brain / Q000378:metabolism; D002417:Cattle; D004198:Disease Susceptibility; D016643:Encephalopathy, Bovine Spongiform / Q000378:metabolism / Q000635:transmission*; D019286:Endopeptidase K / Q000378:metabolism; D005858:Germany; D007150:Immunohistochemistry / Q000662:veterinary; D051379:Mice; D008822:Mice, Transgenic; D008969:Molecular Sequence Data; D008970:Molecular Weight; D016133:Polymerase Chain Reaction / Q000379:methods / Q000662:veterinary; D018621:PrPC Proteins / Q000737:chemistry* / Q000378:metabolism / Q000472:pathogenicity*; D018620:PrPSc Proteins / Q000737:chemistry* / Q000378:metabolism / Q000472:pathogenicity*; D013997:Time Factors
chemical_list: D018621:PrPC Proteins; D018620:PrPSc Proteins; D019286:Endopeptidase K