TACI and BCMA are receptors for a TNF homologue implicated in B-cell autoimmune disease

摘要: B cells are important in the development of autoimmune disorders by mechanisms involving dysregulated polyclonal B-cell activation, production pathogenic antibodies, and co-stimulation autoreactive T cells. zTNF4 (BLyS, BAFF, TALL-1, THANK) is a member tumour necrosis factor (TNF) ligand family that potent co-activator vitro vivo. Here we identify two receptors for demonstrate relationship between disease. Transgenic animals overexpressing lymphoid develop symptoms characteristic systemic lupus erythaematosus (SLE) expand rare population splenic B-Ia lymphocytes. In addition, circulating more abundant NZBWF1 MRL-lpr/lpr mice during onset progression SLE. We have identified TNF receptor members, TACI BCMA, bind zTNF4. Treatment with soluble TACI-Ig fusion protein inhibits proteinuria prolongs survival animals. These findings involvement its SLE as promising treatment disease humans.