The pathology associated with ALS commonly arises from high levels of mis-spliced FUS and TDP-43 mRNAs [145,146], which presumably could overload the NMD machinery due to the elevated rate of aberrant transcripts and result in overproduction of truncated proteins, as suggested by Jaffrey and Wilkinson [147]. The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.