TARDBP and amyotrophic lateral sclerosis: Considering the pivotal role of TDP-43 in maintaining genomic integrity and given its mislocalization or nuclear clearance impairs DNA DSB repair in ALS-affected MNs [11], we examined the level of DSB marker γH2ax in cortical tissues of ALS and sham mice by western blotting which showed ~three-fold higher expression of γH2ax in mutant mice brain samples than in sham controls (Fig. 6a-b).