Spontaneous Generation of Prion Infectivity in Fatal Familial Insomnia Knockin Mice

@article{Jackson2009SpontaneousGO,
  title={Spontaneous Generation of Prion Infectivity in Fatal Familial Insomnia Knockin Mice},
  author={Walker S. Jackson and Andrew W. Borkowski and Henryk Faas and Andrew D Steele and Oliver D. King and Nicki E Watson and Alan Jasanoff and Susan Lindquist},
  journal={Neuron},
  year={2009},
  volume={63},
  pages={438-450}
}
A crucial tenet of the prion hypothesis is that misfolding of the prion protein (PrP) induced by mutations associated with familial prion disease is, in an otherwise normal mammalian brain, sufficient to generate the infectious agent. Yet this has never been demonstrated. We engineered knockin mice to express a PrP mutation associated with a distinct human prion disease, fatal familial insomnia (FFI). An additional substitution created a strong transmission barrier against pre-existing prions… CONTINUE READING
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Prions

  • S. B. Prusiner
  • Proc . Natl . Acad . Sci . USA
  • 1998
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