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12:57
Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
Published on: January 8, 2015
Prion-like disorders: blurring the divide between transmissibility and infectivity
Mimi Cushman1, Brian S Johnson, Oliver D King
1Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, 805b Stellar-Chance Laboratories, 422 Curie Boulevard, Philadelphia, PA 19104, USA.
Journal of Cell Science
|April 2, 2010
Summary
Prions and similar self-templating proteins can spread, causing neurodegenerative diseases like Alzheimer's and Parkinson's. This transmissibility necessitates caution with new cell-based therapies to prevent disease spread.
Area of Science:
- Neuroscience
- Molecular Biology
- Protein Chemistry
Background:
- Prions are self-templating proteins causing transmissible diseases.
- Amyloid formation is not exclusive to prions and is linked to neurodegenerative disorders.
- Proteins like FUS and TDP-43, implicated in neurodegenerative diseases, share prion-like domains.
Purpose of the Study:
- To review advances in understanding the transmissibility of amyloid forms associated with neurodegenerative diseases.
- To explore the potential 'infectious' nature of proteins linked to Alzheimer's, Parkinson's, and Huntington's diseases.
- To reassess the safety and optimization of neuronal graft and stem-cell therapies in light of protein transmissibility.
Main Methods:
- Review of current scientific literature on prion diseases and protein misfolding.
- Analysis of studies demonstrating cell-to-cell spread of amyloid conformers in neurodegenerative conditions.
- Evaluation of the implications of protein transmissibility for therapeutic strategies.
Main Results:
- Amyloid conformers associated with Alzheimer's, Parkinson's, and Huntington's diseases can spread from cell to cell.
- This cell-to-cell spread contributes to the progression of distinct neurodegenerative phenotypes.
- The findings highlight the 'infectious' potential of misfolded proteins in the brain.
Conclusions:
- The transmissibility of amyloid conformers necessitates a re-evaluation of neuronal graft and stem-cell therapies.
- Strategies must be developed to mitigate the risk of transmitting these neurodegenerative agents through therapies.
- Optimizing treatments to overcome transmissible conformers is crucial for patient safety and therapeutic efficacy.
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