Mutations or deletions in TREX1 result in the cytosolic DNA buildup [192], initiating autoimmune responses associated with conditions such as Aicardi-Goutières syndrome (AGS), systemic lupus erythematosus (SLE), familial chilblain lupus, retinal vasculopathy with cerebral leukodystrophy, and renal thrombotic microangiopathy [193]. Here, TREX1 is linked to systemic lupus erythematosus.