An activating NLRC4 inflammasome mutation causes autoinflammation with recurrent macrophage activation syndrome

Abstract

Inflammasomes are innate immune sensors that respond to pathogen- and damage-associated signals with caspase-1 activation, interleukin (IL)-1β and IL-18 secretion, and macrophage pyroptosis. The discovery that dominant gain-of-function mutations in NLRP3 cause the cryopyrin-associated periodic syndromes (CAPS) and trigger spontaneous inflammasome activation… (More)
DOI: 10.1038/ng.3089

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