ZBP1 and systemic lupus erythematosus: To assess whether ZBP1 upregulation functions broadly to drive IFN-dependent pathology, we used another mouse model of type I interferonopathy caused by deficiency in TREX1, a cytosolic 3ʹ–5ʹ DNA exonuclease found to be mutated in people with AGS, familial chilblain lupus (FCL) and systemic lupus erythematosus (SLE)34.