Almost all ALS cases and over half of all cases of FTD are characterized by the cytoplasmic accumulation of SGs containing ubiquitin-positive and Ser409/Ser410 phosphorylated TDP-43 [4,144], an RBP involved in different processes that span from the regulation of RNA metabolism, transport, and translation of specific mRNAs [145,146,147,148,149,150,151,152] to SG formation [151]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.