ADAR2-deficient motor neurons in both AR2 mice and sporadic ALS patients share several characteristics, including slow progressive death of motor neurons except for those innervating extraocular muscles, expression of Q/R site-unedited GluA2, and mislocalization of TDP-4317, 18, 19, 20, 21, 33. Here, ADARB1 is linked to amyotrophic lateral sclerosis.