TARDBP and amyotrophic lateral sclerosis: Chemical induction of ER stress in NSC-34 cells led to increased TDP-43 cleavage (Suzuki et al., 2011), while overexpression of ALS-associated TDP-43A315T and Q331K mutants in Neuro2a cells led to ER stress and activation of a number of UPR stress response pathways, including increased CHOP protein levels, increased nuclear XBP1 and increased activation of ATF6 (Walker et al., 2013).