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Cryopyrin-associated periodic syndrome (CAPS), clinically characterized by neutrophil-rich urticarial rash, is associated with missense mutations in NLRP3. NLRP3 is a pattern recognition receptor in the cytoplasm of cells and structurally related to plant resistance proteins, which detect pathogen- or danger-associated signals, leading to programmed cell death and hyper response at the local site in plants. In mammals, activated NLRP3 forms inflammasome, which orchestrates the early inflammatory process via IL-1β activation, and also cause programmed necrotic cell death termed “pyronecrosis”. However, the mechanistic details are largely unknown.

PW02-025 - Programme necrosis by CAPS-associated NLRP3