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The familial ALS protein FUS promotes DNA repair. (A) A schematic depicting the structure of the FUS protein with ALS missense mutations highlighted. (B) FUS functions to regulate the DNA damage response through interactions with HDAC1 and PARP1. These interactions are important for H2AX phosphorylation (P) and 53BP1-mediated DSB repair. Loss of FUS or ALS-linked mutations leads to defective DSB repair and genomic instability.
