Depletion of TDP-43 from the nucleus and accumulation in cytoplasmic inclusions can be found in autopsy specimens in the majority of ALS cases and in frontotemporal dementia.21, 22, 23 Although TARDBP mutations account for a small percentage of ALS cases, the general presence of this pathology in other ALS types indicates an important role of the protein in stressed neurons and in ALS pathogenesis.21, 23 Thus, we focused experiments on TDP-43 variants linked to fALS as well as pursuing experiments with SOD1 and FUS variants. This evidence concerns the gene FUS and amyotrophic lateral sclerosis.