Figure 1.

Pathophysiology of cryopyrin-associated periodic syndromes (CAPS). Because of a mutation in the NLRP3/cryopyrin gene, there is a constant activation of the regulatory system of Il-1β synthesis and secretion. A first signal activates expression of pro-Il-1β and components of the protein complex inflammasome NLRP3/cryopyrin. A second signal triggers the assembly of the inflammasome, and initiates caspase-1 activation required for the cleavage of pro-Il-1β into active Il-1β