AGS patients show an increased expression of interferon-stimulated genes (ISGs), the so-called “interferon signature”, commonly observed in CD4+ T cells, monocytes, and monocyte-derived macrophages from peripheral blood (Rice et al., 2013; Galli et al., 2018; Aso et al., 2019). This evidence concerns the gene STING1 and Aicardi-Goutieres syndrome.