In a previous work it has been shown that, in monocytes from MKD patients, a key component of the inflammation machinery is NACHT, LRR and PYD domains-containing protein 3 (NALP3) [64]; NALP3 interacts with another protein, pyrin domain (PYD) of apoptosis-associated speck-like protein containing a CARD domain (ASC), constituting the inflammasome platform. Here, NLRP3 is linked to mevalonic aciduria.