Contribution of the inflammasomes to autoinflammatory diseases and recent mouse models as research tools.

Inflammasomes are multiprotein complexes that serve as activating platforms for the enzyme caspase-1 in response to various danger signals. Active caspase-1 can cleave the precursors of the pro-inflammatory cytokines IL-1β and IL-18 and thereby activate them. Deregulation of this cascade caused by mutations in genes coding for inflammasomal components and their interaction partners can lead to severe disease. This review summarizes the contribution of deregulated inflammasomes to the field of autoinflammatory syndromes. In addition, it gives insight into currently available mouse models that are used to study and characterize the role of the inflammasome components in the pathophysiology of these diseases.