Complement regulatory proteins and autoimmunity.
The complement system is known to be involved in autoimmunity at several levels. Activated complement contributes to the inflammatory tissue injury characteristic of many autoimmune disease settings. On the other hand, early components of the classical pathway, including C1q, C4 and C2, are thought to be important for disposing apoptotic cellular autoantigens and/or the induction of B cell tolerance in the bone marrow, and their deficiency is a strong risk factor for systemic autoimmunity. Recent studies using transgenic mice have revealed membrane complement regulatory proteins as important modulators in the pathogenesis and manifestation of autoimmune injury. Available evidence suggests that these regulatory proteins may act to suppress autoimmunity via both complement-dependent and -independent mechanisms.
Song
Wen-Chao
WC
Institute for Translational Medicine and Therapeutics, University of Pennsylvania School of Medicine, Rm 1254 BRBII/III, 421 Curie Blvd, Philadelphia, PA 19104, USA. song@spirit.gcrc.upenn.edu
D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology; D015551:Autoimmunity / Q000276:immunology*; D018958:CD55 Antigens / Q000276:immunology*; D018957:CD59 Antigens / Q000276:immunology*; D003165:Complement System Proteins / Q000276:immunology; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D023421:Models, Animal; D011951:Receptors, Complement / Q000276:immunology*; D017463:Receptors, Complement 3b
D018958:CD55 Antigens; D018957:CD59 Antigens; C081470:Cr1l protein, mouse; D011951:Receptors, Complement; D017463:Receptors, Complement 3b; D003165:Complement System Proteins