Figure 5.

Na_v channel blockers prevent motoneuron death induced by ACM-SOD1^G93A, ACM-SOD1^G86R, and ACM-TDP43^A315T. (A) Flow diagram of experiment. ACMs were applied chronically starting at 4 DIV alone, or together with the Na_v channel blockers mexiletine (25 nM), spermidine (10 μ M), or riluzole (100 nM). Cell survival was assayed at 7 DIV. (B–E) Graphs showing the relative percentage of motoneurons that survived at 7 DIV, after being treated with the diverse Na_v channel blockers and ACM-SOD1^G93A (B), ACM-SOD1^G86R (C), ACM-TDP43^A315T (D), or ACM-SOD1^WT (E), relative to motoneurons from sister cultures treated with control medium (indicated with^*) or with only the ACM (indicated with^#). Values represent means ± s.e.m. from at least 3 independent experiments, analyzed by One-Way ANOVA followed by a Tukey post-hoc test. ^*P < 0.05, ^***P < 0.001 relative to survival with control media at 7 DIV; ^##P < 0.01 and ^###P < 0.001 compared to survival with ALS-causing ACM at 7 DIV.