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Glial Contributions to Lafora Disease: A Systematic Review
Stefania Della Vecchia1, Maria Marchese2, Filippo Maria Santorelli1,2
1Molecular Medicine and Neurogenetics, IRCCS Stella Maris Foundation, Calambrone, 56128 Pisa, Italy.
Biomedicines
|December 23, 2022
Summary
Glial cells, including astrocytes and microglia, play a significant role in Lafora disease (LD) pathogenesis. Targeting these cells may offer new therapeutic strategies for this neurodegenerative disorder.
Area of Science:
- Neuroscience
- Cell Biology
- Pathology
Background:
- Lafora disease (LD) is a fatal neurodegenerative disorder.
- Characterized by polyglucosan body (PB) accumulation in the brain.
- Neuroinflammation is a key feature of LD.
Purpose of the Study:
- Investigate the role of astrocytes and microglia in Lafora disease.
- Systematically review the literature on glial cells in LD.
- Explore potential therapeutic targets in LD.
Main Methods:
- Systematic review adhering to PRISMA guidelines.
- Searches conducted in PubMed, Scopus, and Web-of-Science.
- Independent review by two researchers.
Main Results:
- 35 studies were included in the review.
- Evidence suggests glial cells (astrocytes and microglia) accumulate PBs in LD.
- Glial cells contribute to neurodegeneration and epilepsy in LD.
Conclusions:
- Glial cells are implicated in Lafora disease pathogenesis.
- Targeting glial cells offers potential therapeutic avenues for LD.
- Therapeutic responses may vary due to diverse glial cell phenotypes.
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