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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.

Original publication

DOI

10.3201/eid1810.120792

Type

Journal article

Journal

Emerging infectious diseases

Publication Date

10/2012

Volume

18

Pages

1574 - 1579

Addresses

The Roslin Institute, Easter Bush, Scotland.

Keywords

Brain, Animals, Mice, Inbred C57BL, Humans, Mice, Creutzfeldt-Jakob Syndrome, Prions, PrPSc Proteins, Adult, United States, France, Italy, Netherlands, Female, Male, Young Adult, United Kingdom