In the context of inflammatory autoimmune diseases, such as systemic lupus erythematosus (SLE) or Aicardi-Goutières syndrome (AGS), it is well established that TREX1 deficiency results in aberrant cGAS/STING-mediated type I IFN production, underlining the important IFN-regulatory function of TREX1 (31, 32). This evidence concerns the gene TREX1 and Aicardi-Goutières syndrome.