TARDBP and amyotrophic lateral sclerosis: Although mutations in the TARDDP gene reduce the number of steps to the onset of ALS only by two compared to individuals without mutations, transactive response DNA binding protein-43 (TDP-43), which is encoded by TARDDP, can be found in neuronal inclusions in more than 97% of sALS patients, suggesting a pivotal role of the protein in the pathology of ALS (52).