title: Membrane complement regulatory proteins: insight from animal studies and relevance to human diseases.
abstract: The complement system plays an important role in host defense. However, if not properly regulated, activated complement can also cause significant damage to host tissues. To prevent complement-mediated autologous tissue damage, host cells express a number of membrane-bound complement regulatory proteins. These include decay-accelerating factor (DAF, CD55), membrane cofactor protein (MCP, CD46) and CD59. Recent studies of membrane complement regulatory proteins from various animal species have revealed similarities as well as significant differences from the corresponding human proteins. In this review, we summarize recent advances in this area and contrast the structure, function and tissue distribution of membrane complement regulatory proteins in human and nonprimate mammalian species. We also discuss how the characterization of the animal proteins has provided important clues and might continue to show relevance to the pathogenesis and therapeutics of a number of human diseases.
authors.affiliation: Centre for Experimental Therapeutics and Department of Pharmacology, University of Pennsylvania School of Medicine, 1351 BRBII-III, 421 Curie Blvd., Philadelphia, PA 19104, USA.
authors.forename: T
authors.initials: T
authors.lastname: Miwa
authors.forename: W C
authors.initials: WC
authors.lastname: Song
mesh_terms: D000818:Animals; D015703:Antigens, CD / Q000378:metabolism; D000954:Antigens, Surface; D018958:CD55 Antigens / Q000378:metabolism; D018957:CD59 Antigens / Q000378:metabolism; D003167:Complement Activation; D003165:Complement System Proteins / Q000378:metabolism*; D006168:Guinea Pigs; D006801:Humans; D051916:Membrane Cofactor Protein; D008562:Membrane Glycoproteins / Q000378:metabolism; D008565:Membrane Proteins / Q000378:metabolism*; D051379:Mice; D023421:Models, Animal; D051381:Rats; D011956:Receptors, Cell Surface; D011951:Receptors, Complement / Q000378:metabolism; D017463:Receptors, Complement 3b / Q000378:metabolism; D013552:Swine
chemical_list: D015703:Antigens, CD; D000954:Antigens, Surface; C497857:CD46 protein, human; D018958:CD55 Antigens; D018957:CD59 Antigens; C497860:Cd46 protein, rat; C081470:Cr1l protein, mouse; C513105:Cr1l protein, rat; C497859:Mcp protein, mouse; D051916:Membrane Cofactor Protein; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D011956:Receptors, Cell Surface; D011951:Receptors, Complement; D017463:Receptors, Complement 3b; D003165:Complement System Proteins