NR AWCP
AU Buschmann,A.; Gretzschel,A.; Biacabe,A.G.; Schiebel,K.; Corona,C.; Hoffmann,C.; Eiden,M.; Baron,T.G.M.; Casalone,M.; Tagliavini,F.; Langeveld,J.; Groschup,M.H.
TI Characterization of atypical BSE cases of the H- and L-type in Germany
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions PR-06
PT Konferenz-Poster
AB After the detection of BSE in the UK in 1986, it was assumed that BSE only occurred as a single strain. However, since 2003 BSE cases of two deviant kinds (L-type and H-type) have been reported in France and Italy in animals that were over eight years of age. Therefore we have re-assesed the 27 German BSE cases that were over eight years of age when the disease was diagnosed out of the 389 German cases that have been detected between November 2000 and December 2005. This characterization focused on the determination of the molecular mass (Mr) of the unglycosylated band as well as on the glycoprofile of the accumulated PrPsc by immunoblot analysis. We detected one L-type case which was characterised by a decreased Mr of the unglycosylated PrPsc and a unique glycopattern with almost equal percentages of the di- and monoglycosylated PrPsc fractions as well as one H-type case with a distinctly elevated Mr molecular mass of this smallest PrPsc fraction and an unaltered glycoprofile (using mab L42). Comparative analyses of these two cases together with French and Italian cases of the H- and L-types proved that these two cases indeed belong to the same groups. Both German isolates were inoculated into bovine PrP transgenic mice (Tgbov XV mice). The L-type provoked disease in these mice after very short incubation times of 183 days as compared to 230 days after challenge with classical BSE. In contrast, the H-type has not lead to any clinical symptoms in the mice after 300 days. Interestingly, the characteristic immunoblot profile of the L-type BSE was maintained after passage through Tgbov XV mice. In order to study the pathogenesis of these novel BSE strains in their natural host, transmission studies in 6 months old calves (i.c. route) have been initiated.
AD A. Buschmann, A. Gretzschel, C. Hoffmann, M. Eiden, M.H. Groschup: Friedrich-Loeffler-Institut (FLI), Institute for Novel and Emerging Infectious Diseases, Insel Riems, Germany; A.-G. Biacabe: AFSSA-Lyon, Unite ATNC, Lyon, France; K. Schiebel, T. Baron: Institute for Biochemistry, University Erlangen-Nürnberg; C. Corona, M. Casalone: CEA - Instituto Zooprofilattico di Turino, Turin, Italy; F. Tagliavini: Instituto Nazionale Neurologico Carlo Besta' (INNB), Milano, Italy; J. Langeveld: CIDC-Lelystad, Lelystad, The Netherlands. E-mail: martin.groschup@fli.bund.de
SP englisch
PO Italien