Of relevance to impaired RNA homeostasis, over 95% of ALS patients display pathological accumulation of TDP-43, an RNA-binding protein that binds to 30% of the human transcriptome (Neumann et al., 2006; Freibaum et al., 2010; Ling et al., 2013). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.