title: Constant transmission properties of variant Creutzfeldt-Jakob disease in 5 countries.
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.
authors.affiliation: The Roslin Institute, Easter Bush, Scotland.
authors.forename: Abigail B
authors.initials: AB
authors.lastname: Diack
authors.forename: Diane
authors.initials: D
authors.lastname: Ritchie
authors.forename: Matthew
authors.initials: M
authors.lastname: Bishop
authors.forename: Victoria
authors.initials: V
authors.lastname: Pinion
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Brandel
authors.forename: Stephane
authors.initials: S
authors.lastname: Haik
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Tagliavini
authors.forename: Cornelia
authors.initials: C
authors.lastname: Van Duijn
authors.forename: Ermias D
authors.initials: ED
authors.lastname: Belay
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Gambetti
authors.forename: Lawrence B
authors.initials: LB
authors.lastname: Schonberger
authors.forename: Pedro
authors.initials: P
authors.lastname: Piccardo
authors.forename: Robert G
authors.initials: RG
authors.lastname: Will
authors.forename: Jean C
authors.initials: JC
authors.lastname: Manson
mesh_terms: D000328:Adult; D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology* / Q000635:transmission*; D005260:Female; D005602:France; D006801:Humans; D007558:Italy; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D009426:Netherlands; D018620:PrPSc Proteins / Q000378:metabolism; D011328:Prions / Q000378:metabolism / Q000472:pathogenicity*; D006113:United Kingdom; D014481:United States; D055815:Young Adult
chemical_list: D018620:PrPSc Proteins; D011328:Prions