TREX1 and Aicardi-Goutières syndrome: Malfunctioning of TREX1 has thus been shown to lead to inflammation and autoimmune diseases such as inflammatory myocarditis in Trex1-/- mice [18] and systemic lupus erythematosus (SLE), Aicardi-Goutières syndrome (AGS), retinal vasculopathy, cerebral leukodystrophy, and familial chilblain lupus (FCL) in TREX1-deficient humans [14,19,20].