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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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Replication of prions in differentiated muscle cells
Allen Herbst1, Judd M Aiken1, Debbie McKenzie2
1Centre for Prions and Protein Folding Diseases; Department of Agricultural, Food and Nutritional Sciences; University of Alberta; Edmonton, AB Canada.
Prion
|February 13, 2014
Summary
Prions accumulate in differentiated C2C12 myotubes, not myoblasts. This finding highlights a novel system for robust prion replication and study in non-proliferative cell cultures.
Area of Science:
- Neuroscience
- Cell Biology
- Prion Biology
Background:
- Prion diseases are transmissible neurodegenerative disorders characterized by the misfolding of the prion protein (PrP).
- Previous research indicated that C2C12 myoblasts, a type of muscle precursor cell, are not capable of replicating prions.
- C2C12 cells can differentiate into myotubes, which are non-proliferative, post-mitotic muscle cells.
Purpose of the Study:
- To investigate the capacity of differentiated C2C12 myotubes for prion replication.
- To confirm that C2C12 myoblasts do not accumulate disease-associated prion protein (PrPSc).
- To establish the utility of C2C12 myotube cultures as a model system for studying prion replication.
Main Methods:
- Culturing and differentiating C2C12 myoblasts into myotubes.
- Inoculating differentiated myotubes with prions.
- Assessing prion accumulation and replication using biochemical and cell-based assays.
Main Results:
- C2C12 myoblasts, in their replicative state, do not accumulate PrPSc.
- Differentiated, post-mitotic C2C12 myotubes robustly replicate prions.
- High levels of prion accumulation were observed in the non-proliferative myotube cultures.
Conclusions:
- Non-proliferative C2C12 myotubes provide a competent cellular system for robust prion replication.
- This finding contrasts with the inability of proliferative C2C12 myoblasts to replicate prions.
- C2C12 myotube cultures represent a valuable and effective model for prion disease research.
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