To examine whether methylation-mediated cGAS autoinhibition presents a therapeutic strategy for AGS, we isolated PBMCs from an AGS patient with frameshift mutations c.459dupA and c.695delA on TREX1, resulting in heterozygous Cys154Met and Tyr232Ser mutations, leading to highly elevated lSGs expression (Fig. 7E, F). This evidence concerns the gene CGAS and Aicardi-Goutieres syndrome.