Autophagy is implicated in ALS and in the degradation of misfolded SOD1 and TDP-43.11–13 Autophagy is also dysregulated in cells expressing mutant TDP-43 and in SOD1G93A transgenic mice.13–15 However, whether autophagy is impaired in cells expressing ALS-associated mutant FUS (mFUS) remains unclear. Here, SOD1 is linked to amyotrophic lateral sclerosis.