Cell models of prion infectionDidier Vilette
Unité Mixte de Recherche 1225, INRA, ENVT, 31000 Toulouse, France
Received 23 July 2007; accepted 24 September 2007; published online 27 November 2007
Abstract - Due to recent renewal of interest and concerns in prion diseases, a number of cell systems permissive to prion multiplication have been generated in the last years. These include established cell lines, neuronal stem cells and primary neuronal cultures. While most of these models are permissive to experimental, mouse-adapted strains of prions, the propagation of natural field isolates from sheep scrapie and chronic wasting disease has been recently achieved. These models have improved our knowledge on the molecular and cellular events controlling the conversion of the PrP protein into abnormal isoforms and on the cell-to-cell spreading of prions. Infected cultured cells will also facilitate investigations on the molecular basis of strain identity and on the mechanisms that lead to neurodegeneration. The ongoing development of new cell models with improved characteristics will certainly be useful for a number of unanswered critical issues in the prion field.
Key words: prion diseases / cell models / prion dissemination / apoptosis / PrP protein
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© INRA, EDP Sciences 2008