Arima et al. reported that the causal mutation of NNS is a p.G201V mutation in PSMB8 exon 5 [8] and Liu and co-workers described one patient with the CANDLE syndrome who possessed a homozygous nonsense mutation at position 405 resulting in a C to A change with a protein truncation. This evidence concerns the gene PSMB8 and proteosome-associated autoinflammatory syndrome.