SOD1 and amyotrophic lateral sclerosis: Although several studies indicate that high levels of wild-type FUS are toxic for MNs13–16, and MNn degeneration was demonstrated to be at least in part dependent on toxic gain of function in mouse models of ALS-FUS17,18, astrogliosis and microgliosis were also found in these same mice, as in SOD1 mice and ALS patients, suggesting that non-cell autonomous mechanisms can take place in FUS-mediated pathology, and contribute to disease progression.