Thus, a sufficient amount of unmodified LUBAC remains in unstimulated patient-derived cells, and once stimulated with TNF enough LUBAC can be recruited to Complex I for adequate activation of NF-κB (Fig. 5 G), suggesting that reduced activation of NF-κB is not a common feature of ORAS patients. The gene discussed is NFKB1; the disease is autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.