TARDBP and amyotrophic lateral sclerosis: Conditional ADAR2 knockout mice (ADAR2flox/flox/VAChT-Cre.Fast; AR2) exhibit a progressive ALS phenotype19, 20 resulting from the death of motor neurons due to ADAR2-downregulation, and notably exhibit mislocalization of TDP-43 from the nucleus to the cytoplasm in the ADAR2-lacking motor neurons21.