SETX and amyotrophic lateral sclerosis: However, mutant SOD1 causes less than 1% of ALS cases and the list of mutant proteins found in ALS patients has grown noticeably in the last decade (Marangi and Traynor, 2014), to include a number of proteins related to cell functions as diverse as RNA metabolism (TDP-43, FUS/TLS, Senataxin, Ataxin2, HNRNPA2/B1, ELP3, HNRNPA1), vesicle trafficking (Alsin, FIG4, OPTN, VABP, CHMP2B) and proteasomal function (UBQLN2, VCP).