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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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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8064759/
https://www.ncbi.nlm.nih.gov/pubmed/33851575
http://dx.doi.org/10.7554/eLife.65311