Loss-of-function DNASE1 mutations and lowered DNASE1 expression in diseased kidneys of both individuals with SLE and lupus-prone mice have been described (Fenton et al., 2009; Martinez-Valle et al., 2010; Napirei et al., 2000; Seredkina et al., 2009; Tsukumo and Yasutomo, 2004; Zykova et al., 2010). Here, DNASE1 is linked to systemic lupus erythematosus.