In amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), mutations in transactive response DNA-binding protein 43 (TDP-43) and fused in sarcoma (FUS) cause aberrant RNA splicing, and some of the mis-spliced transcripts need to be degraded through NMD; thus, enhancing NMD activity protects neurons in the cellular models of ALS and FTD (Barmada et al., 2015). Here, TARDBP is linked to frontotemporal dementia.