Prion diseases are characterized by the conversion of the normal cellular prion protein (PrP
C), a glycoprotein that is anchored to the cell membrane by a glycosylphosphatidylinositol
moiety, into an isoform that is protease-resistant (PrP res) and pathogenic. In inherited prion
diseases, mutations in the prion protein (PrP M) engender the conversion of PrP M into PrP
res. We developed a cell model of Gerstmann-Sträussler-Scheinker disease, a
neurodegenerative condition characterized by PrP M-containing amyloid deposits and …