The involvement of EREs in AGS has been supported by the observation that LINE1 loci are hypomethylated in patient-derived fibroblasts (Lim et al., 2015), and extrachromosomal accumulation of LINE1 has been reported to drive type I IFN activation in TREX1-deficient astrocytes that could be rescued by blocking LINE1 RT with a cocktail of RTis (Thomas et al., 2017). This evidence concerns the gene TREX1 and Aicardi-Goutieres syndrome.