SOD1 and amyotrophic lateral sclerosis: Notably, HSF1 has been shown to prolong the survival of a mutant SOD1-ALS mouse model69, while Chen et al., found evidence of a compromised HSF1 response pathway in transgenic TDP-43 mouse models and ALS patients, and showed that a constitutively active form of HSF1 reduced the level of insoluble TDP-43 in mammalian cells26.