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Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
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Prion-like aggregates: infectious agents in human disease

Gunilla T Westermark1, Per Westermark

  • 1Department of Medical Cell Biology and Department of Genetics and Pathology, Uppsala University, Uppsala, Sweden.

Trends in Molecular Medicine
|September 28, 2010
PubMed

Insights

Prion diseases like Creutzfeldt-Jakob disease may be transmissible via misfolded proteins. This study explores if amyloid-associated diseases share similar transmissibility mechanisms, potentially crossing species barriers.

Area of Science:

  • Neuroscience
  • Protein Misfolding Diseases
  • Infectious Diseases

Background:

  • Prion disorders are linked to the 'protein only hypothesis,' involving transmissible, misfolded prion proteins acting as templates.
  • Prion protein misfolding and aggregation resemble amyloid fibril formation seen in common human and animal diseases.
  • Transmissibility has been demonstrated in murine models of amyloidosis, including a model of human AA amyloidosis.

Purpose of the Study:

  • To investigate the potential transmissibility of human prion diseases and common amyloid deposit-associated maladies.
  • To explore the concept of 'seeding' as a mechanism for disease transmission.
  • To examine the possibility of prion-like diseases crossing species barriers.

Main Methods:

  • Comparative analysis of prion protein misfolding with amyloid fibril genesis.
  • Review of existing data on transmissible amyloidosis models in mice.
  • Exploration of theoretical frameworks for inter-species transmission of protein misfolding diseases.

Main Results:

  • The 'protein only hypothesis' provides a framework for prion disease transmission.
  • Amyloid fibril formation shares similarities with prion protein aggregation.
  • Murine models demonstrate transmissibility of amyloidosis, including AA amyloidosis.

Conclusions:

  • Human prion diseases and common amyloid diseases may share transmissible properties.
  • Seeding mechanisms could be involved in the spread of these conditions.
  • Further research is warranted to understand the potential for cross-species transmission.

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