PMCA-generated prions from the olfactory mucosa of patients with Fatal Familial Insomnia cause prion disease in mice

BACKGROUND: Fatal Familial Insomnia (FFI) is a genetic prion disease caused by the D178N mutation in the prion protein gene (PRNP) in coupling phase with methionine at PRNP 129. In 2017, we have shown that the olfactory mucosa (OM) collected from FFI patients contained traces of PrPSc detectable by...

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Veröffentlicht in:eLife
Hauptverfasser: Bistaffa, Edoardo, Marín-Moreno, Alba, Espinosa, Juan Carlos, De Luca, Chiara Maria Giulia, Cazzaniga, Federico Angelo, Portaleone, Sara Maria, Celauro, Luigi, Legname, Giuseppe, Giaccone, Giorgio, Torres, Juan Maria, Moda, Fabio
Format: Artigo
Sprache:Inglês
Veröffentlicht: eLife Sciences Publications, Ltd 2021
Schlagworte:
Medicine
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC8064759/
https://ncbi.nlm.nih.gov/pubmed/33851575
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.7554/eLife.65311
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