NLRP3 inflammasome
Central innate immune sensor driving caspase-1 activation and IL-1β/IL-18 maturation
- Expression
- Overexpressed and hyperactivated
- Evidence level
- established
Role in pathogenesis
The NLRP3 inflammasome is excessively activated in AOSD patients. Upon stimulation by DAMPs and PAMPs through TLRs, NLRP3 recruits ASC adaptor proteins and activates caspase-1, which cleaves pro-IL-1β and pro-IL-18 into bioactive forms. NLRP3, caspase-1, and IL-1β expression are all significantly elevated in PBMCs from AOSD patients. NLRP3 inhibition reduces IL-1β and IL-18 protein expression in AOSD PBMCs.