PLCG2 and agammaglobulinemia: Furthermore, non-mutated NLRP3 activation has been involved in different autoinflammatory syndromes, and, for example, patients with mutations in PLCG2 (autoinflammation and phospholipase Cγ2-associated antibody deficiency and immune dysregulation, APLAID syndrome) present an aberrant cytosolic Ca+2 signaling leading to NLRP3 activation, or patients with mutations in the deubiquitinase OTULIN (otulipenia) result in aberrant IL-1 production by NLRP3 activation (201, 202).