NR AWBO

AU Baron,T.G.M.; Biacabe,A.G.; Bencsik,A.A.; Jacobs,J.G.; Langeveld,J.P.M.; Acutis,P.L.; Polak,M.; Gavier-Widen,D.; Buschmann,A.; Groschup,M.H.; Richt,J.A.

TI Strain variability in bovine atypical TSEs

QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Oral sessions ORAL-24

PT Konferenz-Vortrag

AB Since 2003, some cases of prion diseases in cattle have shown unusual features as assessed by molecular characterization of the protease-resistant prion protein (PrPres) and/or histopathology, when compared to the unique features of BSE described previously. Similar cases have now been recognized in a number of countries, and an overview of the current situation will be presented. Such studies have allowed to refine the molecular definition of such cases using Western blotting, referred as H-type (Biacabe et al., 2004) or L-type (BASE)(Casalone et al., 2004). While a single strain of infectious agent had previously been recognized when BSE was transmitted to a panel of genetically defined inbred wild-type mice, the recent unusual findings raised the question of transmission of prion disease from such unusual isolates. We could show transmission of prion disease from unusual BSE isolates in murine experimental models (including wild-type and transgenic mouse lines). Most importantly, data obtained during the characterization of experimentally infected mice showed different features when compared to those previously described in mice infected with typical BSE isolates. The unusual PrPres molecular features initially described in the brain of cattle by Western blot were maintained following transmission of the agent into mice. In this presentation, the potential origin of such cases, including the possible existence of "sporadic" forms of prion diseases in cattle, will be discussed.

AD Thierry G. M. Baron (t.baron@lyon.afssa.fr), Anne-Gaelle Biacabé, Anna A. Bencsik (a.bencsik@lyon.afssa.fr), Agence Francaise de Sécurité Sanitaire des Aliments (AFSSA), Laboratoire d'Etudes et de Recherches en Pathologie Bovine et Hygiène des Viandes, Unité Agents Transmissibles Non Conventionnels (ATNC), 31 Avenue Tony Garnier, 69364 Lyon cedex 07, France; J. Jacobs, J.P.M. Langeveld: CIDC-Lelystad, Dept. Bacteriology & TSEs, Lelystad, The Netherlands; P.L. Acutis: CEA - Istituto Zooprofilattico del Piemonte, Torino, Italy; M. Polak: NVRI, Dept. Virology, Pulawy, Poland; D. Gavier-Widén: SVA, Upssala, Sweden; A. Buschmann, M.H. Groschup: FLI-INEID, Riems, Greifswald, Germany; J.A. Richt: NADC/USDA, Ames, Iowa, USA. E-mail: t.baron@lyon.afssa.fr

SP englisch

PO Italien

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