In a mouse model of the IFN-related autoimmune condition Aicardi-Goutières Syndrome (AGS), which is mimicked by a deletion of dsDNA-degrading protein 3′ repair endonuclease 1 (Trex1), B cells were shown to be responsible for the development of glomerulonephritis and greatly contributed to disease-related mortality (25). Here, TREX1 is linked to Aicardi-Goutieres syndrome.