TP53BP1 and amyotrophic lateral sclerosis: As in C9orf72-ALS, VCP depletion impairs the recruitment of the DNA repair factor, 53BP1, to sites of DNA damage (Acs et al., 2011; Meerang et al., 2011), suggesting a common theme by which 53BP1-mediated DNA repair is defective in ALS (Fig. 3A).