NR ABXI
AU Bruce,M.E.; Will,R.G.; Ironside,J.W.; McConnell,I.; Drummond,D.; Suttie,A.; McCardle,L.; Chree,A.; Hope,J.; Birkett,C.R.; Cousens,S.N.; Fraser,H.; Bostock,C.J.
TI Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent
QU Nature 1997 Oct 2; 389(6650): 498-501
KI Nature. 1997 Oct 2;389(6650):423. PMID: 9333220 Nature. 1997 Oct 2;389(6650):437-8
PT journal article
AB There are many strains of the agents that cause transmissible spongiform encephalopathies (TSEs) or 'prion' diseases. These strains are distinguishable by their disease characteristics in experimentally infected animals, in particular the incubation periods and neuropathology they produce in panels of inbred mouse strains. We have shown that the strain of agent from cattle affected by bovine spongiform encephalopathy (BSE) produces a characteristic pattern of disease in mice that is retained after experimental passage through a variety of intermediate species. This BSE 'signature' has also been identified in transmissions to mice of TSEs of domestic cats and two exotic species of ruminant, providing the first direct evidence for the accidental spread of a TSE between species. Twenty cases of a clinically and pathologically atypical form of Creutzfeldt-Jakob disease (CJD), referred to as 'new variant' CJD (vCJD), have been recognized in unusually young people in the United Kingdom, and a further case has been reported in France. This has raised serious concerns that BSE may have spread to humans, putatively by dietary exposure. Here we report the interim results of transmissions of sporadic CJD and vCJD to mice. Our data provide strong evidence that the same agent strain is involved in both BSE and vCJD.
MH Animal; Brain/pathology; Cats; Cattle; Creutzfeldt-Jakob Syndrome/*etiology/pathology/transmission; Encephalopathy, Bovine Spongiform/*etiology/pathology/transmission; Glycosylation; Human; Mice; Mice, Inbred C57BL; Prion Diseases/etiology/pathology/transmission; *Prions/chemistry/pathogenicity; Species Specificity; Support, Non-U.S. Gov't; Time Factors
AD Moira E. Bruce (moira.bruce@bbsrc.ac.uk), Irene McConnell, D. Drummond, A. Suttie, A. Chree, Hugh Fraser, Institute for Animal Health, Neuropathogenesis Unit, Ogston Building,West Mains Road, Edinburgh EH9 3JF, UK; R. G. Will, J. W. Ironside, L. McCardle, National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK; J. Hope, C. Birkett, C. J. Bostock, Institute for Animal Health, Compton, Newbury, Berkshire RG20 7NN, UK; Simon Cousens, Department of Epidemiology and Population Science, London School of Hygiene and Tropical Medicine, Keppel Street, London WC1E 7HT, UK
SP englisch
PO England