A link between ADAR1 and type I IFN production in human also came from observations in Aicardi–Goutières syndrome (AGS) [57], a severe autoimmune disease with early onset encephalopathy associated with a high level of IFN-α in the cerebrospinal fluid and upregulated ISG transcription [58,59,60,61]. This evidence concerns the gene ADAR and Aicardi-Goutieres syndrome.