Glial Contributions to Lafora Disease: A Systematic Review
Background: Lafora disease (LD) is a neurodegenerative condition characterized by the accumulation of polyglucosan bodies (PBs) throughout the brain. Alongside metabolic and molecular alterations, neuroinflammation has emerged as another key histopathological feature of LD. Methods: To investigate t...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-12-01
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| Series: | Biomedicines |
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| Online Access: | https://www.mdpi.com/2227-9059/10/12/3103 |
| _version_ | 1797461376206110720 |
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| author | Stefania Della Vecchia Maria Marchese Filippo Maria Santorelli |
| author_facet | Stefania Della Vecchia Maria Marchese Filippo Maria Santorelli |
| author_sort | Stefania Della Vecchia |
| collection | DOAJ |
| description | Background: Lafora disease (LD) is a neurodegenerative condition characterized by the accumulation of polyglucosan bodies (PBs) throughout the brain. Alongside metabolic and molecular alterations, neuroinflammation has emerged as another key histopathological feature of LD. Methods: To investigate the role of astrocytes and microglia in LD, we performed a systematic review according to the PRISMA statement. PubMed, Scopus, and Web-of-Science database searches were performed independently by two reviewers. Results: Thirty-five studies analyzing the relationship of astrocytes and microglia with LD and/or the effects of anti-inflammatory treatments in LD animal models were identified and included in the review. Although LD has long been dominated by a neuronocentric view, a growing body of evidence suggests a role of glial cells in the disease, starting with the finding that these cells accumulate PBs. We discuss the potential meaning of glial PB accumulations, the likely factors activating glial cells, and the possible contribution of glial cells to LD neurodegeneration and epilepsy. Conclusions: Given the evidence for the role of neuroinflammation in LD, future studies should consider glial cells as a potential therapeutic target for modifying/delaying LD progression; however, it should be kept in mind that these cells can potentially assume multiple reactive phenotypes, which could influence the therapeutic response. |
| first_indexed | 2024-03-09T17:18:32Z |
| format | Article |
| id | doaj.art-b5c2afc1883b4bd984bda77a344f7832 |
| institution | Directory Open Access Journal |
| issn | 2227-9059 |
| language | English |
| last_indexed | 2024-03-09T17:18:32Z |
| publishDate | 2022-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomedicines |
| spelling | doaj.art-b5c2afc1883b4bd984bda77a344f78322023-11-24T13:26:51ZengMDPI AGBiomedicines2227-90592022-12-011012310310.3390/biomedicines10123103Glial Contributions to Lafora Disease: A Systematic ReviewStefania Della Vecchia0Maria Marchese1Filippo Maria Santorelli2Molecular Medicine and Neurogenetics, IRCCS Stella Maris Foundation, Calambrone, 56128 Pisa, ItalyNeurobiology, IRCCS Stella Maris Foundation, Calambrone, 56128 Pisa, ItalyMolecular Medicine and Neurogenetics, IRCCS Stella Maris Foundation, Calambrone, 56128 Pisa, ItalyBackground: Lafora disease (LD) is a neurodegenerative condition characterized by the accumulation of polyglucosan bodies (PBs) throughout the brain. Alongside metabolic and molecular alterations, neuroinflammation has emerged as another key histopathological feature of LD. Methods: To investigate the role of astrocytes and microglia in LD, we performed a systematic review according to the PRISMA statement. PubMed, Scopus, and Web-of-Science database searches were performed independently by two reviewers. Results: Thirty-five studies analyzing the relationship of astrocytes and microglia with LD and/or the effects of anti-inflammatory treatments in LD animal models were identified and included in the review. Although LD has long been dominated by a neuronocentric view, a growing body of evidence suggests a role of glial cells in the disease, starting with the finding that these cells accumulate PBs. We discuss the potential meaning of glial PB accumulations, the likely factors activating glial cells, and the possible contribution of glial cells to LD neurodegeneration and epilepsy. Conclusions: Given the evidence for the role of neuroinflammation in LD, future studies should consider glial cells as a potential therapeutic target for modifying/delaying LD progression; however, it should be kept in mind that these cells can potentially assume multiple reactive phenotypes, which could influence the therapeutic response.https://www.mdpi.com/2227-9059/10/12/3103Lafora diseaseastrocytesmicroglianeuroinflammationneurodegenerationepilepsy |
| spellingShingle | Stefania Della Vecchia Maria Marchese Filippo Maria Santorelli Glial Contributions to Lafora Disease: A Systematic Review Biomedicines Lafora disease astrocytes microglia neuroinflammation neurodegeneration epilepsy |
| title | Glial Contributions to Lafora Disease: A Systematic Review |
| title_full | Glial Contributions to Lafora Disease: A Systematic Review |
| title_fullStr | Glial Contributions to Lafora Disease: A Systematic Review |
| title_full_unstemmed | Glial Contributions to Lafora Disease: A Systematic Review |
| title_short | Glial Contributions to Lafora Disease: A Systematic Review |
| title_sort | glial contributions to lafora disease a systematic review |
| topic | Lafora disease astrocytes microglia neuroinflammation neurodegeneration epilepsy |
| url | https://www.mdpi.com/2227-9059/10/12/3103 |
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