Figure 1.

Schematic summarization of our recent studies,⁶ which provided new molecular insights on the role of FUS in nuclear genome maintenance and implications of FUS mutations in ALS. Fused in sarcoma is required for optimal DNA nick ligation in healthy neurons to facilitate efficient oxidative genome damage repair. However, how loss of FUS or its familial mutations in ALS causes DNA nick ligation defects, which could contribute neurodegeneration, is unknown. ALS indicates amyotrophic lateral sclerosis; FUS, fused in sarcoma; ROS, reactive oxygen species.