Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity.

@article{Telling1996EvidenceFT,
  title={Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity.},
  author={Glenn C. Telling and Plero Parchi and Stephen J. DeArmond and Pietro Cortelli and Paola Montagna and Ruth Gabizon and James Mastrianni and Elio Lugaresi and Pierluigi Gambetti and Stanley B Prusiner},
  journal={Science},
  year={1996},
  volume={274 5295},
  pages={2079-82}
}
The fundamental event in prion diseases seems to be a conformational change in cellular prion protein (PrPC) whereby it is converted into the pathologic isoform PrPSc. In fatal familial insomnia (FFI), the protease-resistant fragment of PrPSc after deglycosylation has a size of 19 kilodaltons, whereas that from other inherited and sporadic prion diseases is 21 kilodaltons. Extracts from the brains of FFI patients transmitted disease to transgenic mice expressing a chimeric human-mouse PrP gene… CONTINUE READING
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