Loss of DNA degradation by DNase1L3 causes an autosomal recessive form of SLE with early life onset and high prevalence of nephritis (84), and loss of the Trex1 DNase (85, 86) also leads to constitutive type I production and SLE or AGS, indicating that rapid elimination of aberrant DNA is important for the maintenance of health. Here, TREX1 is linked to Aicardi-Goutieres syndrome.