De novo generation of a PrPSc-like conformation in living cells.

摘要: Conformational conversion of the cellular PrPC protein to PrPSc is a central aspect prion diseases, but how PrP initially converts this conformation remains mystery. Here we show that expressed in yeast cytoplasm, instead endoplasmic reticulum, acquires characteristics PrPSc, namely detergent insolubility and distinct pattern protease resistance. Neuroblastoma cells cultured under reducing, glycosylation-inhibiting conditions produce with same characteristics. We therefore describe what is, our knowledge, first full-length heterologous system, importance reducing deglycosylation conformational transitions, suggest model for initiating events sporadic inherited diseases.