RGFP963 prevented this loss in the SOD1 model, but not in motor neurons expressing the FUS variant; only the combination of RGFP963 and arimoclomol preserved somal, but not dendritic, Hspa8. These data point to differences in how ALS variants affect chaperone expression and to neuroprotective properties of arimoclomol through other mechanisms. Here, FUS is linked to amyotrophic lateral sclerosis.