Article
Nav1.1 haploinsufficiency in excitatory neurons ameliorates seizure-associated sudden death in a mouse model of Dravet syndrome.
Human molecular genetics - 1 Dec 2013
Ogiwara Ikuo, Iwasato Takuji, Miyamoto Hiroyuki, Iwata Ryohei, Yamagata Tetsushi, Mazaki Emi, Yanagawa Yuchio, Tamamaki Nobuaki, Hensch Takao K, Itohara Shigeyoshi, Yamakawa Kazuhiro
Abstract excerpt
Dravet syndrome is a severe epileptic encephalopathy mainly caused by heterozygous mutations in the SCN1A gene encoding a voltage-gated sodium channel Nav1.1. We previously reported dense localization of Nav1.1 in parvalbumin (PV)-positive inhibitory interneurons in mice and abnormal firing of those neurons in Nav1.1-deficient mice. In the present study, we investigated the physiologic consequence of selective...
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