May 14, 2019

Is Targeting of Compensatory Ion Channel Gene Expression a Viable Therapeutic Strategy for Dravet Syndrome?

Loss of function in the Scn1a gene leads to a severe epileptic encephalopathy called Dravet syndrome (DS). Reduced excitability in cortical inhibitory neurons is thought to be the major cause of DS seizures. Here, in contrast, researchers show enhanced excitability in thalamic inhibitory neurons that promotes the nonconvulsive seizures that are a prominent yet poorly understood feature of DS. In a mouse model of DS with a loss of function in Scn1a, reticular thalamic cells exhibited abnormally long bursts of firing caused by the downregulation of calcium-activated potassium SK channels.

The authors claim that this study supports a mechanism in which loss of potassium SK channel activity causes the reticular thalamic neurons to become hyperexcitable and promote nonconvulsive seizures in DS. They propose that reduced excitability of inhibitory neurons is not global in Dravet syndrome and that non-GABAergic mechanisms such as SK channels may be important targets for treatment.

Related News