Disturbances in RNA metabolism in ALS have been keenly studied owing to the discovery that TDP-43 and FUS, which are RNA-binding proteins (RBPs), form neuronal inclusions and harbor mutations that are ALS causative (Neumann et al., 2006; Kwiatkowski et al., 2009; Vance et al., 2009). Here, FUS is linked to amyotrophic lateral sclerosis.