Recombinant oligomeric protein particles composed of the adaptor ASC or the p.D303N mutant form of NLRP3 associated with cryopyrin-associated periodic syndromes (CAPS) stimulate further activation of caspase-1 extracellularly, as well as intracellularly after phagocytosis by surrounding macrophages [40]. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.