Department of Molecular, Cell and Cancer Biology
Genetic Phenomena | Nervous System | Nervous System Diseases | Neuroscience and Neurobiology
Seizures induced by visual stimulation (photosensitive epilepsy; PSE) represent a common type of epilepsy in humans, but the molecular mechanisms and genetic drivers underlying PSE remain unknown, and no good genetic animal models have been identified as yet. Here, we show an animal model of PSE, in Drosophila, owing to defective cortex glia. The cortex glial membranes are severely compromised in ceramide phosphoethanolamine synthase (cpes)-null mutants and fail to encapsulate the neuronal cell bodies in the Drosophila neuronal cortex. Expression of human sphingomyelin synthase 1, which synthesizes the closely related ceramide phosphocholine (sphingomyelin), rescues the cortex glial abnormalities and PSE, underscoring the evolutionarily conserved role of these lipids in glial membranes. Further, we show the compromise in plasma membrane structure that underlies the glial cell membrane collapse in cpes mutants and leads to the PSE phenotype.
Drosophila, glia, neuron, photosensitive epilepsy, sphingolipids
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Copyright © 2018 the Author(s). Published by PNAS. This open access article is distributed under Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND).
DOI of Published Version
Proc Natl Acad Sci U S A. 2018 Sep 18;115(38):E8919-E8928. doi: 10.1073/pnas.1808463115. Epub 2018 Sep 5. Link to article on publisher's site
Proceedings of the National Academy of Sciences of the United States of America
Kunduri G, Turner-Evans D, Konya Y, Izumi Y, Nagashima K, Lockett S, Holthuis J, Bamba T, Acharya U, Acharya JK. (2018). Defective cortex glia plasma membrane structure underlies light-induced epilepsy in cpes mutants. Open Access Articles. https://doi.org/10.1073/pnas.1808463115. Retrieved from https://escholarship.umassmed.edu/oapubs/3608
Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.