Reducing STAU1 levels attenuated in vitro cellular phenotypes, restored SCA2 mouse cerebellar molecular phenotypes, and improved their motor behavior, and normalized the mTOR activity of the unfolded protein response in SCA2 and ALS-TDP-43 mice (9, 15, 16, 17). This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.