NR AXXU
AU Thorne,L.; Terry,L.A.; Jenkins,R.; Everest,S.J.; Chaplin,M.J.; Davis,L.A.; Stack,M.J.
TI The First Case of H-type Bovine Spongiform Encephalopathy (BSE) Identified in Great Britain (GB)
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Natural and Experimental Strains P02.11
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB
Amongst almost 200,000 cases of BSE identified by surveillance worldwide, a small number display molecular profiles of the protease-resistant prion protein that differ from those typically found. These cases have been detected in Europe (Casalone and others, 2004; Biacabe and others, 2004; Buschmann and others, 2006), Japan (Yamakawa and others, 2003) and the USA (Richt and others, 2007). Here we describe the first case of an H-type BSE identified in GB.
BSE with unusual molecular phenotype falls into two groups based on the molecular mass of the unglycosylated PrPres band relative to that of classical BSE, one of higher relative mass (H-type) and the other of lower molecular mass (L-type).
The GB H-type case, a fallen 13 year old Galloway cow, gave a molecular profile indistinguishable from a French H-type case (supplied by Dr T. Baron) when analysed using a modified Bio-Rad TeSeE Western blot and detected using a panel of PrP specific monoclonal antibodies. The unglycosylated band of the H-type cases detected using antibodies Sha31 and 6H4 had a higher molecular mass (~ 20 kD) than those of classical BSE cases and similar to that of classical scrapie. Similar profiles were observed with antibodies P4, L42 and 6H4. P4 and L42 also labelled a band of 6-10 kD that is likely to represent a C-terminally truncated fragment. Four bands from both the French and GB H-type were labelled with the antibody SAF84 and the banding patterns were distinct from those observed with classical BSE. These bands are likely to represent differentially glycosylated forms of a PK cleavage product truncated between amino acids 160-173.
Due to autolysis the brain was unsuitable for further characterisation by immunohistochemistry and PrP gene sequencing. Its age and reported absence of clinical signs are consistent with other cases of H-type BSE.
This first case of H-type BSE was detected in a retrospective and incomplete search for such cases within the population of GB cattle. Full details of that investigation will be reported when complete.
AD L. Thorne, L.A. Terry, R. Jenkins, S.J. Everest, M.J. Chaplin, L.A. Davis, M.J. Stack, Veterinary Laboratories Agency, Molecular Pathogenesis & Genetics, UK
SP englisch
PO Schottland