NR ATEZ

AU Groschup,M.H.; Buschmann,A.; Ziegler,U.; Gretzschel,A.; Hoffmann,C.; Eiden,M.; Weber,A.

TI Biochemical and pathogenetic features of BSE and scrapie infections in ruminants

QU International Conference - Prion 2005: Between fundamentals and society's needs - 19.10.-21.10.2005, Congress Center Düsseldorf - Oral sessions ORAL-04

PT Konferenz-Vortrag

AB The host encoded cellular prion protein (PrPc) plays a crucial role in the pathogenesis and in the infection cycle of TSEs. Following infection, PrPc is converted into the pathological, putatively infectious isoform, designated (PrPsc). However, PrPsc derived from a range of prion strains can have remarkable differences in its biochemical phenotype which may be the basis even for divergent transmission and pathogenesis characteristics. In the course of a large scale TSE surveillance programme 186 TSE-affected sheep were identified in Germany which resembled a) classical scrapie cases as well as b) a novel type, so-called atypical scrapie cases. Similar cases had been found in Norway before and have meanwhile also been confirmed in most European member states. Biochemical and transmission characteristics for these atypical scrapie cases are presented as well as a discriminatory tests to explore any possibility of the presence of BSE infections in natural sheep and goat TSE cases.
The BSE pathogenesis in bovines, i.e. the route of BSE prions from the alimentary tract of cattle to reach the CNS is still little understood. Since January 2003 we are therefore carrying out a BSE pathogenesis study in cattle using fifty six calves which were infected orally with a macerate of BSE positive brainstem (100g per animal) and another 18 animals which serve as mock controls. Live animals are sampled regularly (blood, urine, CSF) and in the course of serial kills selected animals are euthanised at defined time points and autopsied under TSE steril conditions to collect a large number of tissue and bodily fluid samples. Selected samples are inoculated into transgenic mice, that overexpress the bovine PrP (Tgbov XV mice) and which are about 10.000 times more sensitive than wild type mice to cattle derived BSE prions. First results on the BSE pathogenesis in bovines will be presented.

IN Im Rahmen eines großen Überwachungsprogramms wurden in einem leider nicht genannten Zeitraum in Deutschland 186 Scrapie-infizierte Schafe entdeckt, von denen viele dem atypischen Typ Nor98 entsprachen.
Die im Januar 2003 auf der Insel Riems begonnene Pathogenesestudie umfasste 56 Kälber aus einer "Bioherde", die mit jeweils 100 mazeriertem Hirnstamm von BSE-Rindern gefüttert wurden. Weitere 18 Kälber dienten als Mock-Kontrollen. Von allen Tieren werden regelmäßig Blut, Unrin und Cerebrospinalflüssigkeit gesammelt und regelmäßig werden Tiere getötet, um zu verschiedenen Zeitpunkten nach der Inokulation in einer Vielzahl von Organen, Geweben und Körperflüssigkeiten nach PrPsc und Infektiosität zu fahnden.

AD M.H.Groschup, A.Buschmann, U.Ziegler, A.Gretzschel, C.Hoffmann, M.Eiden, A.Weber, Institute for Novel and Emerging Infectious Diseases at the Friedrich-Loeffler-Institute, Insel Riems, Germany

SP englisch

PO Deutschland

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