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

Ina M Vorberg1,2

  • 1German Center for Neurodegenerative Diseases (DZNE e.V.), Sigmund-Freud-Strasse 27, 53127 Bonn, Germany. ina.vorberg@dzne.de.

Viruses
|April 12, 2019
PubMed

Insights

Prions, infectious misfolded proteins, replicate by converting normal proteins. This review explores how different prion strains target cells and cause distinct diseases, focusing on cell biology and replication mechanisms.

Area of Science:

  • Neuroscience
  • Biochemistry
  • Infectious Diseases

Background:

  • Prions are infectious protein aggregates (PrPSc) lacking nucleic acid.
  • They replicate by converting normal cellular prion protein (PrPC) into infectious forms.
  • Prion strains exhibit distinct biological properties and PrPSc structures.

Purpose of the Study:

  • To review current knowledge on prion replication within target cells.
  • To elucidate strain-specific differences in prion cell biology.
  • To understand how prion strains target specific cells and initiate infections.

Main Methods:

  • Literature review of studies on prion cell biology.
  • Analysis of research on prion strain diversity and PrPSc conformers.
  • Examination of findings on subcellular compartments and endocytic pathways in prion replication.

Main Results:

  • Prion strains target distinct brain regions and cause varied disease phenotypes.
  • Prion strain properties are linked to distinct PrPSc quaternary structures.
  • Cellular permissivity to prion strains varies, with unknown reasons for strain-specific refractoriness.

Conclusions:

  • Understanding prion replication requires deeper insight into strain-specific cellular interactions.
  • Endocytic trafficking pathways play a role in prion replication, but strain-specific requirements are unclear.
  • Further research is needed to fully comprehend the mechanisms of prion targeting and infection initiation.
Keywords:
PrPPrionendocytic traffickingstraintransmissible spongiform encephalopathy

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