Homozygous null mutations in DNASE1L3, preventing the expression of this protein which is closely related to DNase1, may also cause a fully-penetrant form of monogenic lupus (176).The role of deficiency of these enzymes in provoking autoimmunity suggests augmenting the activity of DNase may help neutralize errant chromatin and interfere with the pathogenesis of autoimmunity in murine models (177). Here, DNASE1 is linked to systemic lupus erythematosus.