The intronic hexanucleotide (GGGGCC) repeat expansion (HRE) of C9orf72 is the most common genetic cause of ALS in Europe and America [90,91] in which pathogenic roles of non-AUG translation-mediated production of toxic dipeptide repeat (DPR) proteins and sequestration of RBP in nuclear RNA granules have been hypothesized [92]. This evidence concerns the gene C9orf72 and amyotrophic lateral sclerosis.