Inhibitory synaptic transmission is impaired at higher extracellular Ca²⁺ concentrations in Scn1a^+/- mouse model of Dravet syndrome.

ABSTRACT: Dravet syndrome (DS) is an intractable form of childhood epilepsy that occurs in infancy. More than 80% of all patients have a heterozygous abnormality in the SCN1A gene, which encodes a subunit of Na⁺ channels in the brain. However, the detailed pathogenesis of DS remains unclear. This study investigated the synaptic pathogenesis of this disease in terms of excitatory/inhibitory balance using a mouse model of DS. We show that excitatory postsynaptic currents were similar between Scn1a knock-in neurons (Scn1a^+/- neurons) and wild-type neurons, but inhibitory postsynaptic currents were significantly lower in Scn1a^+/- neurons. Moreover, both the vesicular release probability and the number of inhibitory synapses were significantly lower in Scn1a^+/-

SUBMITTER: Uchino K 

PROVIDER: S-EPMC8137694 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature