GRIA2 and amyotrophic lateral sclerosis: AR2 mice exhibit a progressive ALS phenotype (Hideyama et al, 2010; Hideyama & Kwak, 2011), suggesting that RNA editing at the Q/R site of GluA2 is essential for motor neuron health and survival and that the Q/R editing defect in GluA2 is causative to sporadic ALS.