Specifically, TDP-43 dysfunction results in the dysregulated expression of other ALS-associated proteins which also have roles in RNA metabolism, including FUS, ATXN2, and progranulin (PGRN) (Polymenidou et al., 2011; Sephton et al., 2011; Highley et al., 2014). Here, ATXN2 is linked to amyotrophic lateral sclerosis.