Superoxide dismutase 1 mutation caused a ~50% decrease in mitochondrial membrane potential (MMP; measured by TMRM) and in ATP production as compared to controls cells (P < 0.0001; Fig 1D and E). This mitochondria dysfunction was associated with increased oxidative stress; mitochondrial reactive oxygen species (ROS) and total ROS levels were 186% and 250% of control, respectively, (P < 0.0001) in ALS patient‐derived cells relative to control cells (Fig 1F and G). These mitochondrial defects were significantly reduced by treatment with P110 (1 μM/day for 2 days; Fig 1D–G).