Inflammasome formation in familial Mediterranean fever

The complex of pyrin and ASC recruits procaspase, thus forming the pyrin inflammasome that can convert procaspase to active caspase. Active caspase subsequently converts pro-IL-1 and pro-IL-18 to IL-1 and IL-18, respectively. In parallel, caspase also interacts with the pore-forming protein Gasdermin D, which leads to pore formation in cells and subsequent cell membrane rupture (pyroptosis). During this process, cytokines (eg, IL-1 and IL-18) are released into the bloodstream, where they recruit more immune cells and further perpetuate the inflammatory cascade. This inflammasome activation induces an attack of FMF.