C-terminal fragments of approximately 30–35 kDa have been shown to sequester endogenous TDP-43 from the nucleus, resembling the clearance of nuclear TDP-43 seen in people with ALS and FTLD-TDP (Nonaka et al., 2009; Nishimoto et al., 2010; Che et al., 2011, 2015). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.