The first mutations identified as a cause of familial ALS (fALS) mapped to SOD1 (superoxide dismutase 1), whereas mutations in the TARDBP gene, coding for Tar DNA binding protein 43 (TDP43), one of the most common components of protein aggregates in ALS cases, were identified years later [4]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.