In support of this disease association, mutation of κB enhancer sites in the IκBα promoter in mice leads to development of SS, abnormal T cell development and hypersensitivity to septic shock, owing to a defective IκBα feedback loop, leading to constitutive NF-κB activation and deregulated expression of inflammatory mediators [329]. The gene discussed is NFKB1; the disease is synovial sarcoma.