The significance of aberrant RNA metabolism in ALS is increasingly recognized since the discoveries of ALS-causing mutations in many genes that encode RNA-binding proteins (RBPs), including TARDBP, FUS, HNRNPA1, MATR3, TAF15 and TIA1 (Ito et al., 2017). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.