B cell abnormality and autoimmune disorders.
Various abnormalities have been described in B cells from patients with systemic autoimmune diseases such as systemic lupus erythematosus (SLE) and lupus-prone mice. Many of the abnormalities do not appear to be connected with the pathogenesis of the disease. However, various animal models developing lupus-like disease including both spontaneous mutans such as (NZB x NZW)F1 and MRL/lpr and mice generated by transgenic or knockout technology such as Bim-deficient and CD40L-transgenic mice show defect in apoptosis of mature B cells induced by ligation of the B cell antigen receptor (BCR). BCR-mediated apoptosis appears to be involved in deletion of self-reactive B cells. Thus, defect in BCR-mediated apoptosis is a widely observed B cell abnormality in lupus-prone mice and may play a role in the pathogenesis of systemic autoimmune diseases by abrogating deletion of self-reactive B cells.
Tsubata
Takeshi
T
Laboratory of Immunology, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan. tsubata.imm@mri.tmd.ac.jp
D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology*; D001402:B-Lymphocytes / Q000276:immunology* / Q000473:pathology*; D004195:Disease Models, Animal; D006801:Humans