NLRP3 and cryopyrin-associated periodic syndrome: The majority of the NLRP3 mutations in CAPS are missense mutations located in exon 3 of the NLRP3 gene (http://fmf.igh.cnrs.fr/ISSAID/infevers/) which encodes for the NACHT domain, responsible for autorepression of NLRP3 oligomerization by interacting with the LRR domain in healthy cellular condition, and is also involved in the interaction with CARD8 [12, 29].