Structural features of Dnase1L3 responsible for diverse antigenic serum DNA clearance

Autoimmunity develops when extracellular DNA released from dying cells is not cleared from serum. Dnase1 and Dnase1L3 usually digest this extracellular DNA, and mutations in Dnase1L3 often result in autoimmune pathologies such as rheumatoid arthritis, systemic lupus erythematosus, and hypocomplementemia urticarial vasculitis. Often this antigenic DNA is complexed with lipids and other proteins, thus protecting it from Dnase1 digestion. The degradation of common-antigenic forms of serum DNA in complex with lipids and proteins is uniquely attributed to Dnase1L3.