Ontology highlight
ABSTRACT:
SUBMITTER: Jackson WS
PROVIDER: S-EPMC2775465 | biostudies-literature | 2009 Aug
REPOSITORIES: biostudies-literature
Neuron 20090801 4
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. T ...[more]