NR AXKV
AU Delmouly,K.; Casanova,D.; Lehmann,S.; Milhavet,O.
TI Improved Neural Stem Cell Model for Prion Propagation
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Pathology and Pathogenesis P03.169
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB The abnormal prion protein (PrPsc) plays a central role in the transmission of prion diseases. PrPsc is produced by conversion of the cellular isoform of the prion protein (PrPc) which is essential for the pathogenesis of prion diseases. Accumulation of PrPsc in the central nervous system leads to neurodegeneration with neuronal cell death and gliosis. The cellular requirement for the conversion of PrPc, strain specificity and propagation of PrPsc are still unclear. To address these questions and investigate the molecular basis of prion diseases it is a necessity to find new cell culture model or improve those already existent. We recently described that, after differentiation, neural stem cells (NSCs) from the central nervous system are able to convert the cellular isoform of the prion protein into its pathologic scrapie isoform in cell culture. Thus, using this original model we have sustained our investigations to optimise these culture conditions. We hypothesized that changing the culture conditions by modifying a given factor or a combination of factors could increase PrPsc production in NSCs. The first results showed that the infection can be strongly influenced by supplements added to the culture medium. Those supplements enabled neuronal but also astroglial differentiation of NSCs and increased prion propagation and PrPsc production. These results and additional data will add efficiently to our understanding of prion diseases and more importantly allow us to develop more powerful models.
AD K. Delmouly, D. Casanova, S. Lehmann, O. Milhavet, Institut de Genetique Humaine, France
SP englisch
PO Schottland