Recently various mutations in the genes coding for two DNA/RNA binding proteins, TAR DNA binding protein-43 (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS), were identified in familial ALS cases [2], [3], [4], suggesting the molecular mechanisms regulating RNA metabolism could be implicated in familial ALS pathogenesis [1], [5]. This evidence concerns the gene TARDBP and sarcoma.