Functional and structural deficits of the dentate gyrus network coincide with emerging spontaneous seizures in an Scn1a mutant Dravet Syndrome model during development

Neurobiology of Disease
Ming-Shian TsaiShu-Wha Lin

Abstract

Dravet syndrome (DS) is characterized by severe infant-onset myoclonic epilepsy along with delayed psychomotor development and heightened premature mortality. A primary monogenic cause is mutation of the SCN1A gene, which encodes the voltage-gated sodium channel subunit Nav1.1. The nature and timing of changes caused by SCN1A mutation in the hippocampal dentate gyrus (DG) network, a core area for gating major excitatory input to hippocampus and a classic epileptogenic zone, are not well known. In particularly, it is still not clear whether the developmental deficit of this epileptogenic neural network temporally matches with the progress of seizure development. Here, we investigated the emerging functional and structural deficits of the DG network in a novel mouse model (Scn1a(E1099X/+)) that mimics the genetic deficit of human DS. Scn1a(E1099X/+) (Het) mice, similarly to human DS patients, exhibited early spontaneous seizures and were more susceptible to hyperthermia-induced seizures starting at postnatal week (PW) 3, with seizures peaking at PW4. During the same period, the Het DG exhibited a greater reduction of Nav1.1-expressing GABAergic neurons compared to other hippocampal areas. Het DG GABAergic neurons showed altered a...Continue Reading

References

Aug 28, 1989·Brain Research·N C de LanerolleD D Spencer
Jan 1, 1989·Annual Review of Neuroscience·R S Zucker
Mar 1, 1972·Electroencephalography and Clinical Neurophysiology·R J Racine
Jul 1, 1968·The Journal of General Physiology·D O Carpenter, B O Alving
Aug 29, 2000·Epilepsia·D L Burgess, J L Noebels
Oct 3, 2002·Nature Reviews. Neuroscience·Rachel O L Wong, Anirvan Ghosh
Jun 28, 2003·Science·Juan José GarridoBénédicte Dargent
May 5, 2006·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Sebastian BrauchiRamon Latorre
Mar 10, 2007·Brain : a Journal of Neurology·Louise A HarkinIngrid E Scheffer
Jun 1, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Ikuo OgiwaraKazuhiro Yamakawa
Aug 3, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Christoph Schmidt-HieberJosef Bischofberger
Jul 16, 2008·Molecular and Cellular Neurosciences·Amandine DuflocqMarc Davenne
Jan 2, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Andrea Lorincz, Zoltan Nusser
Apr 28, 2009·Nature Neuroscience·Bruce P Bean
Aug 12, 2009·Frontiers in Molecular Neuroscience·Charles R Tessier, Kendal Broadie
Sep 19, 2009·American Journal of Medical Genetics. Part a·Daria RivaSilvana Franceschetti
Oct 12, 2010·Seminars in Cell & Developmental Biology·Christophe LeterrierHelene Vacher
Mar 16, 2011·Gene Expression Patterns : GEP·Hsiang-Po HuangI-Shing Yu
Apr 8, 2011·Epilepsia·Masako SakauchiTateki Fujiwara
Aug 22, 2012·Proceedings of the National Academy of Sciences of the United States of America·Christine S CheahWilliam A Catterall
Mar 26, 2013·The Journal of Clinical Investigation·Franck KalumeWilliam A Catterall
Oct 25, 2013·Genes, Brain, and Behavior·A R MillerJ A Kearney
Jan 24, 2014·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Yu-Chao LiuCheng-Chang Lien
Jul 16, 2014·Proceedings of the National Academy of Sciences of the United States of America·Chao TaiWilliam A Catterall

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Citations

Jan 30, 2016·ACS Chemical Neuroscience·Jo SourbronPeter A M de Witte
Aug 2, 2017·The Journal of Comparative Neurology·Kenneth W JohnsonJimcy Platholi
Jun 20, 2018·Frontiers in Pharmacology·Aliesha GriffinScott C Baraban
Dec 7, 2020·Brain : a Journal of Neurology·Nicola Specchio, Paolo Curatolo
Dec 11, 2020·Neurobiology of Disease·Wanqi Wang, Wayne N Frankel
Jun 19, 2021·Neurobiology of Disease·Nina MiljanovicHeidrun Potschka
Aug 3, 2021·Frontiers in Pharmacology·Shih-Yin HoHorng-Huei Liou

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