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Highly infectious prions are not directly neurotoxic

Iryna Benilova1, Madeleine Reilly1, Cassandra Terry1

  • 1Medical Research Council Prion Unit at University College London (UCL), UCL Institute of Prion Diseases, London W1W 7FF, United Kingdom.

Proceedings of the National Academy of Sciences of the United States of America
|September 9, 2020
PubMed

Insights

Pure prions, infectious agents causing fatal neurodegenerative diseases, are not directly toxic to neurons. This finding decouples prion propagation from neurotoxicity, challenging previous assumptions about prion disease mechanisms.

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Infectious Diseases

Background:

  • Prions cause fatal neurodegenerative diseases in humans and animals.
  • These diseases involve misfolded prion protein assemblies.
  • Prions are assumed to be directly neurotoxic.

Purpose of the Study:

  • To directly test the neurotoxicity of pure infectious prions.
  • To investigate the relationship between prion infectivity and toxicity.
  • To explore the mechanisms underlying prion-induced neurodegeneration.

Main Methods:

  • Development of methods for obtaining exceptionally pure prions from mouse brain.
  • Correlation of defined prion structures with infectivity.
  • Testing the toxicity of pure prions on cultured neurons.
  • Treatment of prion homogenates with sodium lauroylsarcosine.

Main Results:

  • Exceptionally pure, intact infectious prions demonstrated no direct neurotoxicity.
  • Brain homogenates from prion-infected mice were highly toxic to neurons.
  • Sodium lauroylsarcosine treatment eliminated toxicity without affecting prion infectivity.

Conclusions:

  • Prion infectivity and neurotoxicity can be mechanistically uncoupled.
  • The direct neurotoxic effect of prions is questionable.
  • This research opens new avenues for understanding and treating prion diseases.
Keywords:
neurodegenerationneurotoxicityprions

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