Breaching self-tolerance by targeting the gatekeeper

Abstract

Loss-of-function mutations in DNaseL13, the enzyme that restricts the amount of microparticle-associated DNA, cause SLE in humans and mice. In this issue of JEM, Hartl et al. (2021. J. Exp. Med. https://doi.org/10.1084/jem.20201138) uncover a reduction in plasma DNASE1L3 enzymatic activity due to the presence of autoantibodies in patients with nonfamilial SLE.