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NLR family of receptors are important members of the innate immunity that respond to exogenous pathogens and endogenous danger signals. Activation of NLR leads to formation of a multimolecular complex called an inflammasome. This complex is used to drive the processing and release of pro-inflammatory cytokines IL-1β and IL-18. Defects in regulation of an inflammasome activation underlie the development of a series of autoinflammatory diseases such as cryopyrin-associated periodic syndromes, familial Mediterranean fever and others. In this review we discuss forms of pathology associated with mutations and gene polymorphism of inflammasomes NLRP3, NLRP1, NLRС2 and NLRP12.

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