Interestingly, gain of function mutations in MDA5 associated with human immune disorders such as Aicardi-Goutières syndrome, have been shown to render MDA5 more tolerant to the irregular AIRs dsRNA structures generated upon ADAR1 A-to-I editing and inducing an aberrant antiviral response (133). This evidence concerns the gene IFIH1 and Aicardi-Goutières syndrome.