Studies of RNA-binding proteins TAR DNA binding protein of 43 kDa (TDP-43) and fused in sarcoma (FUS) were given an extra dimension when these proteins were identified as causative factors for a number of degenerative diseases, primarily amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) (reviewed in 1). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.