The association of mutations in TDP43 and fused in sarcoma (FUS)/translocated in liposarcoma (TLS), two RNA-binding proteins with striking structural and functional similarities [62], as well as ATXN2, which itself is an RNA binding protein, in ALS [31], suggests a prominent role for RNA binding proteins and RNA-dependent processes in the disease. Here, ATXN2 is linked to amyotrophic lateral sclerosis.