Mutations in the SUMO conjugation motifs, p.R137H, p.P651S, p.E690K, and p.E692K, cause CAPS and in p. D90Y, p.E206G, and R556X, an unspecified autoinflammatory syndrome thus suggesting that sumoylation defects may cause disease by lowering the NLRP3 activation threshold. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.