Although TDP-43 mislocalizes from the nucleus to the cytoplasm in the primary neuronal culture exposed to excitotoxicity [119] and may have a modulatory role in ADAR1-mediated A-to-I RNA editing in the cell lines [125], no studies have demonstrated aberrant RNA editing at the Q/R site in GluA2 in familial ALS carrying TARDBP mutations. Here, TARDBP is linked to amyotrophic lateral sclerosis.