Defective cortex glia plasma membrane structure underlies light-induced epilepsy in cpes mutants
Authors
Kunduri, GovindTurner-Evans, Daniel
Konya, Yutaka
Izumi, Yoshihiro
Nagashima, Kunio
Lockett, Stephen
Holthuis, Joost
Bamba, Takeshi
Acharya, Usha
Acharya, Jairaj K.
UMass Chan Affiliations
Department of Molecular, Cell and Cancer BiologyDocument Type
Journal ArticlePublication Date
2018-09-18Keywords
Drosophilaglia
neuron
photosensitive epilepsy
sphingolipids
Genetic Phenomena
Nervous System
Nervous System Diseases
Neuroscience and Neurobiology
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Show full item recordAbstract
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.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
DOI
10.1073/pnas.1808463115Permanent Link to this Item
http://hdl.handle.net/20.500.14038/40800PubMed ID
30185559Related Resources
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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).Distribution License
http://creativecommons.org/licenses/by-nc-nd/4.0/ae974a485f413a2113503eed53cd6c53
10.1073/pnas.1808463115
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Except where otherwise noted, this item's license is described as 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).