CAPS are a group of inherited autoinflammatory syndromes that represent the clinical spectrum of different mutations of the CIAS1 gene (cold induced autoinflammatory syndrome) that encodes a protein called cryopirine (NALP3), a component of the inflammatory agent that activates IL-1alpha when the cell receives the danger signal. The gene discussed is NLRP3; the disease is cryopyrin-associated periodic syndrome.