This model successfully recapitulates multiple hallmark pathological features of ALS, including a distinct dyskinesia phenotype, global TDP‐43 pathology, MN degeneration, gliosis, severe muscle atrophy with fibrosis, TMEM106B accumulation, and increased SOD1 and APOE4 levels. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.