OR11-004 - IL-1, IL-18 and cell death in NLRP3 driven disease
نویسندگان
چکیده
Introduction Missense mutations in cryopyrin (NLRP3) result in a hyperactive inflammasome that drives overproduction of the pro-inflammatory cytokines interleukin-1b (IL-1b) and IL-18. Mice expressing mutations associated with familial cold autoinflammatory syndrome (FCAS) or MuckleWells syndrome (MWS) exhibit severe, spontaneous inflammation, early death, and hyperresponsiveness to stimuli in vitro. Abrogating IL-1 signaling either genetically or pharmacologically results in modest improvement of life expectancy in murine CAPS, but clearly indicates a role for players in addition to IL-1b.
منابع مشابه
Divergence of IL-1, IL-18, and cell death in NLRP3 inflammasomopathies.
The inflammasome is a cytoplasmic multiprotein complex that promotes proinflammatory cytokine maturation in response to host- and pathogen-derived signals. Missense mutations in cryopyrin (NLRP3) result in a hyperactive inflammasome that drives overproduction of the proinflammatory cytokines IL-1β and IL-18, leading to the cryopyrin-associated periodic syndromes (CAPS) disease spectrum. Mouse l...
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Introduction Mast cells, key effector cells of allergic and innate immune responses, have recently been reported to be an important source of IL-1ß in patients with autoinflammatory conditions such as cryopyrin-associated-periodic-fever syndromes (CAPS). CAPS patients show IL-1beta-driven systemic inflammation together with non-histamine dependent urticarial rash, which are caused by activating...
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