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EnglishThe precise molecular mechanism of how misfolded α-synuclein (α-Syn) accumulates and spreads in synucleinopathies is still unknown. Here, we show the role of the cellular prion protein (PrPC) in mediating the uptake and the spread of recombinant α-Syn amyloids. The in vitro data revealed that the presence of PrPC fosters the higher uptake of α-Syn amyloid fibrils, which was also confirmed in vivo in wild type (Prnp +/+) compared to PrP knock-out (Prnp -/-) mice. Additionally, the presence of α-Syn amyloids blocked the replication of scrapie prions (PrPSc) in vitro and ex vivo, indicating a link between the two proteins. Indeed, whilst PrPC is mediating the internalization of α-Syn amyloids, PrPSc is not able to replicate in their presence. This observation has pathological relevance, since several reported case studies show that the accumulation of α-Syn amyloid deposits in Creutzfeldt-Jakob disease patients is accompanied by a longer disease course.
alpha-Synuclein Amyloids Hijack Prion Protein to Gain Cell Entry, Facilitate Cell-to-Cell Spreading and Block Prion Replication / Aulić, Suzana; Masperone, Lara; Narkiewicz, Joanna; Isopi, Elisa; Bistaffa, Edoardo; Ambrosetti, Elena; Pastore, Beatrice; De Cecco, Elena; Scaini, Denis; Zago, Paola; Moda, Fabio; Tagliavini, Fabrizio; Legname, Giuseppe. - In: SCIENTIFIC REPORTS. - ISSN 2045-2322. - 7:1(2017), pp. 1-12. [10.1038/s41598-017-10236-x]
| Titolo: | alpha-Synuclein Amyloids Hijack Prion Protein to Gain Cell Entry, Facilitate Cell-to-Cell Spreading and Block Prion Replication | |
| Autori: | Aulić, Suzana; Masperone, Lara; Narkiewicz, Joanna; Isopi, Elisa; Bistaffa, Edoardo; Ambrosetti, Elena; Pastore, Beatrice; De Cecco, Elena; Scaini, Denis; Zago, Paola; Moda, Fabio; Tagliavini, Fabrizio; Legname, Giuseppe | |
| Rivista: | ||
| Data di pubblicazione: | 2017 | |
| Volume: | 7 | |
| Fascicolo: | 1 | |
| Pagina iniziale: | 1 | |
| Pagina finale: | 12 | |
| Numero di Articolo: | 10050 | |
| Digital Object Identifier (DOI): | http://dx.doi.org/10.1038/s41598-017-10236-x | |
| URL: | www.nature.com/srep/index.html | |
| Appare nelle tipologie: | 1.1 Journal article |
File in questo prodotto:
| File | Descrizione | Tipologia | Licenza | |
|---|---|---|---|---|
| s41598-017-10236-x.pdf | Versione Editoriale (PDF) |  | Open Access Visualizza/Apri | |
| 41598_2017_10236_MOESM1_ESM.pdf | Supplementary material | Versione Editoriale (PDF) | | Open Access Visualizza/Apri |
