Deficiency in these ADAR1 functions due to 11 mutations affect either the catalytic domain or RNA binding (RB) loops (Wright and Vissel, 2012) and underlie the pathogenesis of autoinflammatory diseases, such as the type I interferonopathies Aicardi-Goutières syndrome. The gene discussed is ADAR; the disease is Aicardi-Goutières syndrome.