Mutations in TREX1 cause a spectrum of type I IFN-dependent autoinflammatory and autoimmune phenotypes including Aicardi–Goutières syndrome (AGS), familial chilblain lupus, retinal vasculopathy with cerebral leukodystrophy (RVCL) and systemic lupus erythematosus (SLE)5, 6, 7, 8, 9. The gene discussed is TREX1; the disease is Aicardi-Goutieres syndrome.