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Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
Published on: January 8, 2015
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
Summary
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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