While the cause of the disease in sporadic cases is mostly unknown, the majority of patients present with similar neuropathological lesions: the RNA-binding protein TAR DNA-binding protein 43 (TDP-43) was identified as the major component of the ubiquitin-positive neuronal inclusion bodies observed in nearly all ALS patients (~97 %) and patients of the FTLD-TDP subtype (~45 %) [3, 68, 90]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.