NR AXJO
AU Bishop,M.; Barron,R.; McConnell,I.; Head,M.; Ironside,J.; Will,R.; Manson,J.
TI Sporadic CJD Strain Classification by Transmission to Human Transgenic Mice
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Oral Abstracts FC2.3
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Vortrag
AB
Background: The publication by Parchi et al (Classification of sporadic Creutzfeldt-
Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol, 1999: 46 (2), 224-33) is widely used to group sporadic Creutzfeldt-Jakob disease (sCJD) cases into six different subtypes.
Aims/Objectives: Our goal was to determine whether these sCJD groups would show distinctive transmission properties to laboratory mice and therefore be described as sCJD strains, or whether the PrP-Sc type or codon 129 genotype showed dominance in the transmission results.
Methods: CNS material was prepared from typical cases of 'Parchi' types and inoculated into transgenic mice expressing human prion protein with genotype variation at codon 129.
Results: Examination of the transmission properties (incubation period, lesion profile, and immunocytochemical and/or biochemical detection of PrP-Sc) has shown: 1) Six types of sCJD (MM1, MV1, VV1, MM2, MV2, VV2) give different phenotypes on transmission to transgenic mice. 2) Data suggest that PrP-Sc type has a more dominant effect on the lesion profile than codon 129 genotype. 3) Material containing a dominance of type 2 Valine/PrP-Sc is the most efficient at disease transmission.
Conclusions: This sCJD transmission dataset will subsequently allow for classification of novel atypical sCJD cases.
This work is partly funded by the HUMTRANS NeuroPrion project.
AD M. Bishop, M. Head, J. Ironside, R. Will, UK National CJD Surveillance Unit, UK; R. Barron, I. McConnell, J. Manson, Roslin Institute / Neuropathogenesis Unit, UK
SP englisch
PO Schottland