Furthermore, a direct test of ADAR deamination in Ig SHM in vivo could be achieved in either ADAR1 deficient Aicardi-Goutières Syndrome (AGS) patients (76, 77) or catalytically inactive ADAR1 mouse strains, such as Adar1E861A/E861A Ifih1−/− (78). Here, ADAR is linked to Aicardi-Goutieres syndrome.