PW02-025 - Programme necrosis by CAPS-associated NLRP3
نویسندگان
چکیده
Introduction 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 pathogenor 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-1b activation, and also cause programmed necrotic cell death termed “pyronecrosis”. However, the mechanistic details are largely unknown.
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Introduction Cryopyrin-Associated Periodic Syndromes (CAPS) are a group of rare, inherited, autoinflammatory diseases involved of Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS) and Neonatal Onset Multisystem Inflammatory Disease (NOMID) (also called Chronic Infantile Neurologic Cutaneous Articular, or CINCA, Syndrome. The responsible gene NLRP3 (nucleotide-binding d...
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