In lupus nephritis, which is the most serious complication of SLE, down-regulation of renal DNase I has been demonstrated to result in reduced chromatin fragmentation and deposition of extracellular chromatin-IgG complexes in the glomerular basement membranes in individuals producing IgG anti-chromatin antibodies41,42. Here, DNASE1L3 is linked to systemic lupus erythematosus.