FUS, whose defects are associated with ~ 5% of familial ALS and ~ 1% of sporadic ALS cases due to loss of its nuclear localization and subsequent cytosolic aggregation, has been shown to facilitate DNA ligation during the final step of oxidative damage repair [315] and to be required for DSB repair by NHEJ and HR [316, 317]. Here, FUS is linked to amyotrophic lateral sclerosis.