Interestingly, a nucleus-enriched RBP, TDP-43, translocates to cytoplasmic SGs in response to some stimuli and has been identified as a core component of pathogenic protein aggregation, called “TDP-43 proteinopathy” in amyotrophic lateral sclerosis (ALS), Huntington’s disease, Alzheimer’s disease, and FTD (Arai et al., 2006; Hasegawa et al., 2008; Tada et al., 2012; Ling et al., 2013). Here, TARDBP is linked to amyotrophic lateral sclerosis.