Title
Defective cortex glia plasma membrane structure underlies light-induced epilepsy in cpes mutants
UMMS Affiliation
Department of Molecular, Cell and Cancer Biology
Publication Date
2018-09-18
Document Type
Article
Disciplines
Genetic Phenomena | Nervous System | Nervous System Diseases | Neuroscience and Neurobiology
Abstract
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.
Keywords
Drosophila, glia, neuron, photosensitive epilepsy, sphingolipids
Rights and Permissions
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
10.1073/pnas.1808463115
Source
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
Journal/Book/Conference Title
Proceedings of the National Academy of Sciences of the United States of America
Related Resources
PubMed ID
30185559
Repository Citation
Kunduri, Govind; Turner-Evans, Daniel; Konya, Yutaka; Izumi, Yoshihiro; Nagashima, Kunio; Lockett, Stephen; Holthuis, Joost; Bamba, Takeshi; Acharya, Usha; and Acharya, Jairaj K., "Defective cortex glia plasma membrane structure underlies light-induced epilepsy in cpes mutants" (2018). Open Access Articles. 3608.
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.
Included in
Genetic Phenomena Commons, Nervous System Commons, Nervous System Diseases Commons, Neuroscience and Neurobiology Commons