In humans, the AGS phenotype is associated with mutations in eight other different genes, namely, TREX1 (9), RNASEH2A, RNASEH2B, RNASEH2C (10), SAMHD1 (11), ADAR1 (12), LSM11 (13), and RNU7-1 (14), involved either in nucleic acid metabolism or in the constitutive activation of their sensors, which, in turn, activates the cellular innate IFN-α-mediated immune response (9, 15). The gene discussed is IFNA1; the disease is Aicardi-Goutieres syndrome.