In this study, we assessed if GRT-X, whichhas been demonstrated simultaneously to activate TSPO and Kv7.2/3, may prevent MN loss and ROS production in dissociatedrat primary spinal cord MN cultures (VSCNs) and rat SCOC treated withACM collected from human and mouse astrocytes with ALS/FTD-linkedmutations in SOD1 and TARDBP genes. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.