Molecular biology and pathology of prion strains in sporadic human prion diseases

@article{Gambetti2010MolecularBA,
  title={Molecular biology and pathology of prion strains in sporadic human prion diseases},
  author={Pierluigi Gambetti and Ignazio Cali and Silvio Notari and Qingzhong Kong and Wen-Quan Zou and Witold K Surewicz},
  journal={Acta Neuropathologica},
  year={2010},
  volume={121},
  pages={79-90}
}
Prion diseases are believed to propagate by the mechanism involving self-perpetuating conformational conversion of the normal form of the prion protein, PrPC, to the misfolded, pathogenic state, PrPSc. One of the most intriguing aspects of these disorders is the phenomenon of prion strains. It is believed that strain properties are fully encoded in distinct conformations of PrPSc. Strains are of practical relevance to human prion diseases as their diversity may explain the unusual heterogeneity… CONTINUE READING

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