Vasoactive intestinal peptide-expressing interneurons are impaired in a mouse model of Dravet syndrome.

ABSTRACT: Dravet Syndrome (DS) is a severe neurodevelopmental disorder caused by pathogenic loss of function variants in the gene SCN1A which encodes the voltage gated sodium (Na⁺) channel subunit Nav1.1. GABAergic interneurons expressing parvalbumin (PV-INs) and somatostatin (SST-INs) exhibit impaired excitability in DS (Scn1a^+/-) mice. However, the function of a third major class of interneurons in DS - those expressing vasoactive intestinal peptide (VIP-IN) -is unknown. We recorded VIP-INs in brain slices from Scn1a^+/-mice and wild-type littermate controls and found prominent impairment of irregular spiking (IS), but not continuous adapting (CA) VIP-INs, in Scn1a^+/- mice. Application of the Nav1.1-specific toxin Hm1a rescued

SUBMITTER: Goff KM 

PROVIDER: S-EPMC6629374 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature