In addition to TDP-43, FUS/TLS, another DNA/RNA binding protein, was recently identified as a causative gene in a subset of ALS [69, 70]; increased FUS immuno-reactivity in FTLD subtypes constitute a new group that combines the neuropathology with undefined histology and with intermediate neurofilament inclusions [32]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.