By decreasing ER stress and reducing p-eIF2α, ATF4, CHOP, and caspases, targeting STAU1 could ameliorate proteostasis, ribostasis, and aberrant SG phenotypes in diseases caused by ATXN2, TDP-43, and C9ORF72 mutations as well as other disease gene mutations or sporadic forms of neurodegenerative diseases. Here, ATXN2 is linked to neurodegenerative disease.