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Constant Transmission Properties of Variant Creutzfeldt-Jakob Disease in 5 Countries
Authors:Abigail B. Diack   Diane Ritchie   Matthew Bishop   Victoria Pinion   Jean-Philippe Brandel   Stephane Haik   Fabrizio Tagliavini   Cornelia Van Duijn   Ermias D. Belay   Pierluigi Gambetti   Lawrence B. Schonberger   Pedro Piccardo   Robert G. Will   Jean C. Manson
Abstract:Variant Creutzfeldt-Jakob disease (vCJD) has been reported in 12 countries. We hypothesized that a common strain of agent is responsible for all vCJD cases, regardless of geographic origin. To test this hypothesis, we inoculated strain-typing panels of wild-type mice with brain material from human vCJD case-patients from France, the Netherlands, Italy, and the United States. Mice were assessed for clinical disease, neuropathologic changes, and glycoform profile; results were compared with those for 2 reference vCJD cases from the United Kingdom. Transmission to mice occurred from each sample tested, and data were similar between non-UK and UK cases, with the exception of the ranking of mean clinical incubation times of mouse lines. These findings support the hypothesis that a single strain of infectious agent is responsible for all vCJD infections. However, differences in incubation times require further subpassage in mice to establish any true differences in strain properties between cases.
Keywords:Prion   strain   variant Creutzfeldt-Jakob disease   transmissible spongiform encephalopathy   Creutzfeldt-Jakob disease   transmission properties   CJD   vCJD   TSE   BSE   prions   United States   the Netherlands   France   Italy   United Kingdom   USA   UK
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