In 95% of ALS cases, the neuropathological signature of the disease is represented by the appearance of TDP-43 aggregates in the cytoplasm.51 In a previous work, TDP-43 was found in the cytoplasm of primary fibroblasts carrying TARDBP c*4G>A and p.A382T mutations.52 To test whether G376D fibroblasts are also characterized by this phenotype, we performed an immunofluorescence assay using an antibody able to recognize the C-terminal region of the TDP-43 protein. Here, TARDBP is linked to amyotrophic lateral sclerosis.