ADARB1 and amyotrophic lateral sclerosis: Accordingly, hypoediting of GRIA2/Gria2 as the main substrate of ADAR2 in the findings of ALS patients and mouse models could be both the triggering event for TDP-43 cleavage with subsequent cytoplasmic aggregation (as mentioned in chapter 4; Yamashita et al., 2017) and the consequence of reduced ADAR2 expression due to nuclear TDP-43 depletion and cytoplasmic aggregation.