Subsequently, Tanaka et al., identified somatic NLRP3 mutations in 69.2% (18/26) of mutation-negative CINCA/NOMID patients, estimating a level of mosaicism ranging from 4.2% to 35.8%; all these somatic mutations induced an increased ASC-dependent NF-κB activation (103). The gene discussed is NFKB1; the disease is CINCA syndrome.