Likewise, in cellular and animal models of ALS/FTD (TDP-43 and C9orf72) and SCA2 (ATXN2-Q127), decreasing STAU1 by RNAi or genetic interaction reverses molecular markers of ER stress, defective autophagy, protein aggregates, neuronal death and glial activation, and improves the behavioral motor phenotype of SCA2 mice [17–19]. Here, TARDBP is linked to amyotrophic lateral sclerosis.