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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
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A New Cell Model for Investigating Prion Strain Selection and Adaptation
Alexandra Philiastides1, Juan Manuel Ribes2, Daniel Chun-Mun Yip3
1MRC Prion Unit at UCL, UCL Institute of Prion Diseases, 33 Cleveland Street, London W1W7FF, UK. a.philiastides@prion.ucl.ac.uk.
Viruses
|September 25, 2019
Summary
Prion diseases involve misfolded prion proteins causing neurodegeneration. Researchers adapted a prion strain to new cells, expanding its host range and creating a model for studying prion adaptation.
Area of Science:
- Neuroscience
- Molecular Biology
- Infectious Diseases
Background:
- Prion diseases are fatal neurodegenerative disorders caused by misfolded prion proteins.
- Prion strains exhibit distinct phenotypes, but the mechanisms of host-strain interaction and adaptation remain unclear.
- Cellular factors influence susceptibility to specific prion strains, impacting disease progression.
Purpose of the Study:
- To investigate the expansion of prion strain repertoire in cell models.
- To develop a cellular model for studying prion strain selection and adaptation.
- To analyze the characteristics of cell-adapted prion strains.
Main Methods:
- Selection of prion-susceptible subclones (PME) from Me7-refractory PK1 neuroblastoma cells.
- Infection of PME clones with distinct prion strains (Me7 and RML).
- Assessment of protease resistance and host range of adapted prion strains.
Main Results:
- Successfully selected PK1 subclones (PME) highly susceptible to the Me7 prion strain.
- Me7-infected PME clones exhibited greater protease resistance than RML-infected PME clones, suggesting distinct strains.
- Passage in PME cells extended the host range of the Me7 prion strain.
Conclusions:
- PME clones provide a valuable cell model for studying prion strain selection and adaptation.
- Cellular adaptation can alter prion strain properties, including host range.
- Understanding these mechanisms is crucial for developing therapeutic strategies against prion diseases.
