NR AOSI
AU Faucheux,B.A.; Haik,S.; Sazdovic,V.; Brandel,J.P.; Duyckaerts,C.; Vital,A.; Laplanche,J.L.; Deslys,J.P.; Dormont,D.; Hauw,J.J.
TI Neuronal loss in the cerebellum of patients with sporadic Creutzfeldt-Jakob disease
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - PG-19
PT Konferenz-Poster
AB
The mechanisms involved in neurodegeneration are still poorly understood in human prion diseases, although neuronal death is a central component of the neuropathological pattern. Neurodegeneration varies according to prion disease forms, brain regions and neuronal populations.
In the cerebellum of patients with sporadic Creutzfeldt-Jakob disease (n=42; age-range=40-84 years), we investigated the density of granule cells (estimated with a computer-assisted image analysis system), some markers of neurodegeneration and neuropathological lesions (spongiosis, gliosis). We also studied the location and amount of the prion protein deposits as shown by immuno-histochemistry of tissue sections. We determined by Western blotting the characteristics of proteinase K resistant prion protein (PrPres type) present in tissue homogenates of the cerebellum. The polymorphism at codon 129 of the PRNP gene was deduced from genomic DNA sequencing. The severity of neuronal loss was associated with the PrPres type (p<0.0001), gliosis (p<0.01), spongiosis (p<0.001), and patient's genotype at codon 129.
Our findings clearly indicate relationships between some characteristics of the host, the neuropathological data, and the type of PrPres present in the tissue. Other determinants remain to be elucidated.
This work was carried out with the support of grants from GIS 'Infections à prions' (F127), INSERM-ATC 'Prions', PHRC (AOM96-117), and EC 'Human TSE' (BMH4-CT98-6003).
AD B.A. Faucheux, S. Haik, V. Sazdovic, J.P. Brandel, C. Duyckaerts, J.J. Hauw, INSERM U360, Lab Neuropathologie R.Escourolle, Hopital de la Salpetriere, Paris, France; A. Vital, Service de Neuropathologie, Hopital Pellegrin, Bordeaux, France; J.L. Laplanche, Service de Biochimie-Biologie Moleculaire, Hopital Lariboisiere, Paris, France; J.P. Deslys, D. Dormont, Service de Neurovirologie, DRM/DSV, CEA, Fontenay-aux-Roses, France
SP englisch
PO Deutschland