Figure 1. Normal DSB repair kinetics in fibroblasts from FUS^R521H ALS fibroblasts.

(A–C) DSBs were quantified as γH2AX foci in G1-arrested ALS-FUS^R521H patient and unaffected sibling (Control) fibroblasts. Cells were DMSO-treated, non-irradiated, or treated as indicated with either (A) 4 μM CPT for 45 min, (B) 20 μM etoposide for 30 min, or (C) 2 Gy IR. γH2AX foci were counted in CENPF-negative cells at the indicated time points after treatment. Data are the average (±SEM) of three independent experiments.