Article
NaV1.1 and NaV1.6 selective compounds reduce the behavior phenotype and epileptiform activity in a novel zebrafish model for Dravet Syndrome.
PloS one - 1 Jan 2020
Weuring Wout J, Singh Sakshi, Volkers Linda, Rook Martin B, van 't Slot Ruben H, Bosma Marjolein, Inserra Marco, Vetter Irina, Verhoeven-Duif Nanda M, Braun Kees P J, Rivara Mirko, Koeleman Bobby P C
Abstract excerpt
Dravet syndrome is caused by dominant loss-of-function mutations in SCN1A which cause reduced activity of Nav1.1 leading to lack of neuronal inhibition. On the other hand, gain-of-function mutations in SCN8A can lead to a severe epileptic encephalopathy subtype by over activating NaV1.6 channels. These observations suggest that Nav1.1 and Nav1.6 represent two opposing sides of the neuronal balance between...
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