Abnormalities in the intracellular nucleic acid sensing machinery TREX1 and other critical players including RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR1 and IFIH1 causes AGS, a monogenic interferonopathy [26, 27]. The gene discussed is IFIH1; the disease is Aicardi-Goutieres syndrome.