Besides these genes, less frequent mutated genes such as ATXN-2, VCP, PGRN, and DCTN1 may concur with C9ORF72 in ALS [60,61,62,63], while potentiating alterations of cell-clearing systems, as discussed below [82,83,84,85] (Figure 2). This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.