Strikingly, the cytoplasmic accumulation of TDP-43 in ALS and FTLD brain cortex, was also found to be associated with decreased levels of its human ortholog CHD2, thereby indicating that aberrant chromatin remodeling might be involved in ALS (Berson et al., 2017) (Figure 6). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.