Several studies have established an association between genetic and protein processing defects in the NF-κB signaling pathway downstream of TNFa in human autoimmune and inflammatory diseases, such as systemic lupus erythematosus (SLE) (5), Sjögren’s syndrome (6), Crohn’s disease (7), ulcerative colitis (8) and rheumatoid arthritis (RA) (9). The gene discussed is TNF; the disease is systemic lupus erythematosus.