Expression of wild-type and V210l mutant prion protein in human neuroblastoma cells

The conversion of the host-encoded prion protein (PrPc) into the insoluble, protease-resistant isoform (PrPsc) is the main pathogenic mechanism of tra nsmissible spongiform encephalopathies. They are fatal neurodegenerative di sorders, which in human occur as sporadic, inherited or familial forms. The se last forms are linked to insert or point mutations of PrPc which may fac ilitate the spontaneous conversion into PrPsc. We have established stably t ransfected human neuroblastoma cells (SH-SY5Y) expressing mutant V2701, or wild-type PrPc. Both proteins were expressed and attached to the cell surfa ce. The mutation in position 210 did not after the biochemical properties o f the protein in comparison with the wild-type protein nor induced any conf ormational changes similar to those observed in PrPsc. (C) 1999 Elsevier Sc ience Ireland Ltd. All rights reserved.