Lupus patients exhibit increased levels of ATP, which results in activation of the inflammasome and the consequent release of cytokines associated with disease pathogenesis.52, 53 To avoid ATP-induced pathological effects, CD39 catalyzes the phosphohydrolysis of ATP into adenosine, which is a potent immune regulator of cells that express A2 and A3 receptors, such as lymphocytes.54 Here, ENTPD1 is linked to systemic lupus erythematosus.