Because ADAR1 inhibits IFN signaling pathways, ADAR1 mutations are associated with type I IFN diseases with elevated ISG expression, including Aicardi-Goutières syndrome (AGS), dyschromatosis symmetrical hereditaria (DSH), bilateral striatal necroptosis (BSN), and spastic paraplegia (10). This evidence concerns the gene ADAR and dyschromatosis symmetrica hereditaria.