Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2018 Jul;75(14):2557-2574.
doi: 10.1007/s00018-018-2823-y. Epub 2018 May 14.

Cellular Mechanisms Responsible for Cell-To-Cell Spreading of Prions

Affiliations
Review

Cellular Mechanisms Responsible for Cell-To-Cell Spreading of Prions

Didier Vilette et al. Cell Mol Life Sci. .

Erratum in

Abstract

Prions are infectious agents that cause fatal neurodegenerative diseases. Current evidence indicates that they are essentially composed of an abnormally folded protein (PrPSc). These abnormal aggregated PrPSc species multiply in infected cells by recruiting and converting the host PrPC protein into new PrPSc. How prions move from cell to cell and progressively spread across the infected tissue is of crucial importance and may provide experimental opportunity to delay the progression of the disease. In infected cells, different mechanisms have been identified, including release of infectious extracellular vesicles and intercellular transfer of PrPSc-containing organelles through tunneling nanotubes. These findings should allow manipulation of the intracellular trafficking events targeting PrPSc in these particular subcellular compartments to experimentally address the relative contribution of these mechanisms to in vivo prion pathogenesis. In addition, such information may prompt further experimental strategies to decipher the causal roles of protein misfolding and aggregation in other human neurodegenerative diseases.

Keywords: Exosomes; Extracellular vesicles; Multivesicular bodies; Prion diseases; Protein aggregation.

Similar articles

See all similar articles

Cited by 9 articles

See all "Cited by" articles

References

    1. Mol Neurodegener. 2012 Aug 24;7:42 - PubMed
    1. Brain. 2016 Dec;139(Pt 12):3187-3201 - PubMed
    1. Biol Chem. 1998 Jun;379(6):655-66 - PubMed
    1. FEBS Lett. 1997 Aug 18;413(2):282-8 - PubMed
    1. Neurobiol Aging. 2014 Aug;35(8):1792-800 - PubMed

LinkOut - more resources

Feedback