Interestingly, even if TARDBP gene mutations account only for a small percentage of fALS cases, TDP-43 expression levels and subcellular localization are altered in almost all ALS patients, with TDP-43 toxic cytoplasmic aggregates (“TDP-43 proteinopathy”) observed in approximately 97 % of all ALS patients. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.