Mutations in the human ADAR1 gene result in the autoimmune disease, Aicardi-Goutières syndrome, while the whole-body knockout mice of Adar1 results in embryonic death due to massive apoptosis and aberrant interferon induction, which can be rescued to live birth by ablating the RLRs, Mavs or Mda5 (melanoma differentiation-associated protein 5) [69,70,71]. Here, IFIH1 is linked to Aicardi-Goutieres syndrome.