For instance, Adar1E861A/E861A mice are lethal, while Adar1W197A/W197A mice exhibit brain malformation—a key clinical feature in patients with AGS (Liddicoat et al., 2015; Nakahama et al., 2021); similarly, the R779H mutation in MDA5 can cause AGS or SLE in different individuals (Rice et al., 2014; Van Eyck et al., 2015). The gene discussed is IFIH1; the disease is Aicardi-Goutieres syndrome.