Figure 2.

Neuronal excitability and therapeutic development. Ion channel modulators have been investigated for efficacy in ALS. Riluzole inhibits glutamate release, enhances glutamate uptake, blocks voltage-dependent sodium channels, antagonizes NMDA receptors and inhibits GABA uptake. Perampanel exerts effects via AMPA receptors, retigabine and ezogabine through potassium channels and mexiletine and flecainide via sodium channels (see text for clinical trial results). These ion channel modulators showed several lines of evidence.