NEK7 and CINCA syndrome: Previously reported NLRP3 mutations have been shown to affect interactions between NLRP3 and its endogenous regulator NEK7 [10, 27]; the p.G755R and p.G755A mutations, which cause CINCA, increase the affinity between NLRP3 and NEK7 [24, 25], while the hypomorphic NLRP3 missense mutation p.D946G binds with less avidity to NEK7 than to the WT protein [26].