The developmental evolution of the seizure phenotype and cortical inhibition in mouse models of juvenile myoclonic epilepsy

The GABAA receptor (GABAAR) α1 subunit mutation, A322D, causes autosomal dominant juvenile myoclonic epilepsy (JME). Previous in vitro studies demonstrated that A322D elicits α1(A322D) protein degradation and that the residual mutant protein causes a dominant-negative effect on wild type GABAARs. He...

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Bibliographic Details
Main Authors: Fazal Arain, Chengwen Zhou, Li Ding, Sahar Zaidi, Martin J. Gallagher
Format: Article
Language:English
Published: Elsevier 2015-10-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996115001904