A fly study links C9orf72 repeat products in glial cells to neurodegeneration
Original source
Glial cell-intrinsic and non-cell autonomous toxicity in a Drosophila C9orf72 neurodegeneration model. (opens in a new tab)Compass summarised this from the study's abstract.
Study details
- Peer review
- Preprint, not yet peer reviewed
Related topics
In a Drosophila model, glial cells producing C9orf72 repeat products showed toxicity, with the GR dipeptide repeat and full G4C2 repeat causing the strongest direct effects. The full G4C2 repeat also caused measurable loss of nearby neurons and activated an endogenous retrovirus. These findings point to effects from diseased glial cells as well as neurons, but do not change treatment for people with ALS or frontotemporal dementia.
Why this matters
The study suggests that glial cells may contribute to C9orf72-related neurodegeneration through both direct damage and effects on nearby neurons. This could help researchers investigate disease mechanisms, but the results were obtained in flies and do not show that targeting these pathways benefits people with ALS.
Limitations and context
This is a bioRxiv preprint using engineered, cell-specific expression in fruit flies, not a clinical study in people. The findings need confirmation in other models and human disease before their relevance to treatment can be established.