NR AXVG
AU Ritchie,D.L.; Brown,P.; Williams,L.; Gibson,S.; Lowrie,S.; LeGrice,M.; Will,R.G.; Kreil,T.R.; Abee,C.R.; Ironside,J.W.
TI Clinical, Neuropathological and Biochemical Features of Sporadic and Variant Creutzfeldt-Jakob Disease in the Squirrel Monkey (Saimiri Sciureus)
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Natural and Experimental Strains P02.17
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB
Background: The use of experimental animal models in prion diseases has a long and successful history, providing valuable information on issues such as disease pathogenesis and agent strain. The squirrel monkey (Saimiri sciureus) has been shown to be highly susceptible to experimental challenge with human prion disease, yet little information is available on the phenotype of different human prion strains in this species.
Objectives: Investigations on the clinical, neuropathological and biochemical features of squirrel monkeys experimentally challenged with either sporadic or variant CJD were carried out to establish if strain characteristics are maintained after transmission in this model.
Materials and methods: Brain homogenates from sporadic or variant CJD patients were inoculated into the frontal cortex of squirrel monkeys. Animals were kept under constant clinical surveillance. At post-mortem, formalin fixed CNS tissue was taken for neuropathological and immunohistochemical analysis with frozen CNS tissue taken for the biochemical detection of PrPres .
Results: Clinical presentation was similar in both sporadic and variant CJD challenged animals; however, clinical features were more severe in sporadic CJD challenged animals with a shorter disease duration. Neuropathological analysis showed two distinct patterns of spongiform change and PrP deposition, mirroring the neuropathological features in humans, although amyloid plaques were absent in both sporadic and variant challenged animals. Western blot analysis for PrPres showed a single PrPres isotype in animals challenged with variant CJD resembling that observed in human variant CJD. In all animals challenged with sporadic CJD a single isotype resembling human sporadic CJD type 1 was observed.
Conclusion: Neuropathological and biochemical analysis have shown that sporadic and variant CJD can be distinguished in the squirrel monkey and that many of the strain characteristics that define these agents are conserved after transmission in this model.
AD D.L. Ritchie, S. Lowrie, M. LeGrice, R.G. Will, J.W. Ironside, University of Edinburgh, National CJD Surveillance Unit, UK; P. Brown, National Institutes of Health, Bethesda, USA; L. Williams, C. Abee, University of Texas MD Anderson Cancer Center, Department of Veterinary Sciences, USA; S. Gibson, University of South Alabama, Department of Comparative Medicine, USA; T.R. Kreil, Baxter Bioscience, Global Pathogen Safety, Austria
SP englisch
PO Schottland