Because ADAR2-lacking AHCs in the AR2res mice express edited GluA2 and hence Ca2+-impermeable AMPA receptors, these results indicate that the NPC and the nucleo-cytoplasmic transport system are altered in ADAR2-deficient AHCs of sporadic ALS patients in a manner dependent on Ca2+ influx. Here, GRIA2 is linked to amyotrophic lateral sclerosis.