The most prominent examples are mutations in Three-prime Repair Exonuclease 1 (TREX1) or Sam domain and HD domain 1 (SAMHD1), that are associated with the rare autoinflammatory disease Aicardi-Goutieres syndrome (AGS) characterized by an exaggerated type I IFN response (van Montfoort et al., 2014). This evidence concerns the gene TREX1 and Aicardi-Goutières syndrome.