A conspicuous number of RNA binding proteins (RBPs) have been shown to be associated with amyotrophic lateral sclerosis (ALS) or other neuromuscular diseases: for example, mutations in RBPs such as hnRNPA1 and A2B1, TDP-43, and FUS are associated with ALS (Table 1) (Kabashi et al., 2008; Kim et al., 2013; Qiu et al., 2014). Here, TARDBP is linked to amyotrophic lateral sclerosis.