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Viruses 2019, 11(4), 334; https://doi.org/10.3390/v11040334

All the Same? The Secret Life of Prion Strains within Their Target Cells

1
German Center for Neurodegenerative Diseases (DZNE e.V.), Sigmund-Freud-Strasse 27, 53127 Bonn, Germany
2
Rheinische Friedrich-Wilhelms-Universität Bonn, 53127 Bonn, Germany
Received: 27 February 2019 / Revised: 2 April 2019 / Accepted: 5 April 2019 / Published: 9 April 2019
(This article belongs to the Special Issue Deciphering the Molecular Targets of Prion and Prion-Like Strains)
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Abstract

Prions are infectious β-sheet-rich protein aggregates composed of misfolded prion protein (PrPSc) that do not possess coding nucleic acid. Prions replicate by recruiting and converting normal cellular PrPC into infectious isoforms. In the same host species, prion strains target distinct brain regions and cause different disease phenotypes. Prion strains are associated with biophysically distinct PrPSc conformers, suggesting that strain properties are enciphered within alternative PrPSc quaternary structures. So far it is unknown how prion strains target specific cells and initiate productive infections. Deeper mechanistic insight into the prion life cycle came from cell lines permissive to a range of different prion strains. Still, it is unknown why certain cell lines are refractory to infection by one strain but permissive to another. While pharmacologic and genetic manipulations revealed subcellular compartments involved in prion replication, little is known about strain-specific requirements for endocytic trafficking pathways. This review summarizes our knowledge on how prions replicate within their target cells and on strain-specific differences in prion cell biology. View Full-Text
Keywords: Prion; transmissible spongiform encephalopathy; strain; PrP; endocytic trafficking Prion; transmissible spongiform encephalopathy; strain; PrP; endocytic trafficking
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Vorberg, I.M. All the Same? The Secret Life of Prion Strains within Their Target Cells. Viruses 2019, 11, 334.

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