Neurodevelopmental defects in Dravet syndromeScn1a+/-mice: selective rescue of behavioral alterations but not seizures by targeting GABA-switch
Lara Pizzamiglio,
Fabrizio Capitano,
Evgeniia Rusina
et al.
Abstract:Dravet syndrome (DS) is a developmental and epileptic encephalopathy (DEE) caused by mutations of the SCN1A gene and characterized by seizures, motor disabilities and cognitive/behavioral deficits, including autistic traits. The relative role of seizures and neurodevelopmental defects in disease progression is not clear yet. A delayed switch of GABAergic transmission from excitatory to inhibitory (GABA-switch) has been reported in models of DS, but its effects on the phenotype have not been investigated. In th… Show more
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