NLRP3 and autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation: Canonical activation of the NLRP3-inflammasome by adding ATP or nigericin after LPS priming resulted in an equal formation of intracellular ASC specks in monocytes and similar release of IL-1β in samples from healthy donors, APLAID patients, and CAPS patients (Fig. 4e).