In 2001, nod-like receptor protein 3 (NLRP3) mutation was discovered to cause a group of rare inherited diseases characterized by recurrent episodes of inflammation.1 Since then, we have learned that NLRP3 initiates pro-inflammatory signaling through the formation of a protein complex called the inflammasome, and disease-driving mutations potentiate this pathway.2 Activation of NLRP3 triggers its oligomerisation, which in turn allows for the recruitment and clustering of the ASC inflammasome adapter and the zymogen protease, caspase-1. This evidence concerns the gene NLRP3 and hereditary disease.