Mutations in TREX1 in humans are associated with the autoimmune and autoinflammatory disorders (Kavanagh et al., 2008; Crow and Rehwinkel, 2009) Aicardi-Goutières syndrome (AGS), familial chilblain lupus (FCL), systemic lupus erythematosus (SLE), and retinal vasculopathy with cerebral leukodystrophy (RVCL). Here, TREX1 is linked to systemic lupus erythematosus.