Article
Preferential expression of SCN1A in GABAergic neurons improves survival and epileptic phenotype in a mouse model of Dravet syndrome.
Journal of molecular medicine (Berlin, Germany) - 1 Dec 2023
Ricobaraza Ana, Bunuales Maria, Gonzalez-Aparicio Manuela, Fadila Saja, Rubinstein Moran, Vides-Urrestarazu Irene, Banderas Julliana, Sola-Sevilla Noemi, Sanchez-Carpintero Rocio, Lanciego Jose Luis, Roda Elvira, Honrubia Adriana, Arnaiz Patricia, Hernandez-Alcoceba Ruben
Abstract excerpt
The SCN1A gene encodes the alpha subunit of a voltage-gated sodium channel (Nav1.1), which is essential for the function of inhibitory neurons in the brain. Mutations in this gene cause severe encephalopathies such as Dravet syndrome (DS). Upregulation of SCN1A expression by different approaches has demonstrated promising therapeutic effects in preclinical models of DS. Limiting the effect to inhibitory neurons...
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