The presence of Q/R site-unedited GluA2 due to downregulation of ADAR2 has been observed in motor neurons of patients with ALS carrying the FUSP525L mutation [144], and reduced ADAR2 activity due to ADAR2 mislocalization or binding with poly-PR and widespread reduction in RNA editing has been found in the motor neurons and iPSC-derived motor neurons of patients with ALS carrying C9orf72 with enhanced HRE [145,146]. The gene discussed is ADARB1; the disease is amyotrophic lateral sclerosis.