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

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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Bibliographic Details
Main Authors: Edoardo Bistaffa, Alba Marín-Moreno, Juan Carlos Espinosa, Chiara Maria Giulia De Luca, Federico Angelo Cazzaniga, Sara Maria Portaleone, Luigi Celauro, Giuseppe Legname, Giorgio Giaccone, Juan Maria Torres, Fabio Moda
Format: Article
Language:English
Published: eLife Sciences Publications Ltd 2021-04-01
Series:eLife
Subjects:
fatal familial insomnia
olfactory mucosa
protein misfolding cyclic amplification
transmission studies
neurodegeneration
prion
Online Access:https://elifesciences.org/articles/65311

Internet

https://elifesciences.org/articles/65311

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