Specifically, we found that (i) ALS patient‐derived fibroblasts carrying SOD1, FUS1, or TDP‐43 mutations all exhibit a significantly increased Drp1‐recruitment to the mitochondria, increased mitochondrial fragmentation, and subsequent mitochondrial dysfunction; and these were all greatly reduced by P110. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.