SETX and amyotrophic lateral sclerosis: RNA binding proteins, and mutations in these proteins, appear to play a central role in ALS, including Tar DNA binding protein (TDP-43) [3–5], FUS [6–9], Rho Guanine nucleotide exchange factor (ARHGEF28) [10–13], mutated superoxide dismutase 1 (SOD1) [14, 15], senataxin (SETX) [16–18] and others (see [19–22] for reviews).