ADAR and Aicardi-Goutieres syndrome: Complete deficiency of Adar1 or its p150 isoform in mice results in the elevated ISG levels in embryos and mortality during embryonic days 11.5–12.5, potentially explaining the absence of AGS patients with ADAR1−/− or ADAR1p150−/p150− mutations (Hartner et al., 2004; Wang et al., 2004; Ward et al., 2011).