TARDBP and amyotrophic lateral sclerosis: As TDP-43 pathology, such as cytoplasmic aggregation, modification and phosphorylation, is the critical pathological feature of ALS and FTD [1, 5], we next examined the outcomes of knockdown of Chop on TDP-43 pathology in treated rNLS8 mice by assessing the levels of overall soluble and insoluble TDP-43 protein, TDP-43 fragments, and pTDP-43.