The latter has been described in ALS (14, 15) as the outcome of prolonged ER stress due to the accumulation of misfolded proteins such as, for instance, transactive response DNA-binding protein 43 (TDP43) (16), fused in sarcoma (FUS) (17), or superoxide dismutase type 1 (SOD1) (18). Here, TARDBP is linked to amyotrophic lateral sclerosis.