Mutations in genes coding for proteins involved in DNA/RNA metabolism, such as fused in sarcoma/translocated in liposarcoma (FUS/TLS, which we will refer to hereafter as FUS) [5], [6], [7], and the 43 kDa transactive response-DNA binding protein (TDP43) [8], [9], [10], [11], have emerged as a leading cause of ALS [12] and other motor neuron diseases [13]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.