Using primary culture models of three forms of ALS caused by mutations in TARDPB, FUS, and SOD1, we have highlighted the differences in the effect of these variants on the expression of HSPs, specifically the stress-inducible isoform HSPA1A and the constitutively expressed HSC, HSPA8, as well as the effect of drugs that potentially alter their expression. Here, SOD1 is linked to amyotrophic lateral sclerosis.