title: Complement in autoimmune diseases.
abstract: The complement system is an ancient and evolutionary conserved element of the innate immune mechanism. It comprises of more than 20 serum proteins most of which are synthesized in the liver. These proteins are synthesized as inactive precursor proteins which are activated by appropriate stimuli. The activated forms of these proteins act as proteases and cleave other components successively in amplification pathways leading to exponential generation of final effectors. Three major pathways of complement pathways have been described, namely the classical, alternative and lectin pathways which are activated by different stimuli. However, all the 3 pathways converge on Complement C3. Cleavage of C3 and C5 successively leads to the production of the membrane attack complex which is final common effector. Excessive and uncontrolled activation of the complement has been implicated in the host of autoimmune diseases. But the complement has also been bemusedly described as the proverbial "double edged sword". On one hand, complement is the final effector of tissue injury in autoimmune diseases and on the other, deficiencies of some components of the complement can result in autoimmune diseases. Currently available tools such as enzyme based immunoassays for functional assessment of complement pathways, flow cytometry, next generation sequencing and proteomics-based approaches provide an exciting opportunity to study this ancient yet mysterious element of innate immunity.
authors.affiliation: Pediatric Allergy and Immunology Unit, Dept. of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India.
authors.forename: Pandiarajan
authors.initials: P
authors.lastname: Vignesh
authors.affiliation: Pediatric Allergy and Immunology Unit, Dept. of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India. Electronic address: rawatamit@yahoo.com.
authors.forename: Amit
authors.initials: A
authors.lastname: Rawat
authors.affiliation: Pediatric Allergy and Immunology Unit, Dept. of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India.
authors.forename: Madhubala
authors.initials: M
authors.lastname: Sharma
authors.affiliation: Pediatric Allergy and Immunology Unit, Dept. of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India.
authors.forename: Surjit
authors.initials: S
authors.lastname: Singh
mesh_terms: D001327:Autoimmune Diseases / Q000235:genetics / Q000276:immunology*; D003167:Complement Activation; D003165:Complement System Proteins / Q000032:analysis* / Q000172:deficiency / Q000276:immunology*; D006801:Humans; D007113:Immunity, Innate; D037601:Mannose-Binding Lectin / Q000172:deficiency / Q000235:genetics
chemical_list: C445619:MBL2 protein, human; D037601:Mannose-Binding Lectin; D003165:Complement System Proteins
keywords: ANCA-associated vasculitides; Alternative pathway; Antiphospholipid syndrome; Autoimmunity; Classical pathway; Complement; Henoch Schonlein purpura; Systemic lupus erythematosus