Clinical aspects of the complement system

Hiroshi Tsukamoto, Takahiko Horiuchi

Research output: Contribution to journalReview article

6 Citations (Scopus)

Abstract

The complement system consists of more than 30 proteins and has 3 types of activation pathways: classical, lectin and alternative pathways. The complement system not only has a role in innate immunity but also works as an antibody-dependent effecter to eliminate pathogens. It is useful to measure serum levels of CH50, C3 and C4 in patients with immune-mediated diseases. While increased levels of CH50 are associated with non-specific inflammation, decreased levels of CH50 in combination with normal or decreased levels of C3 and C4 are associated with specific immune-mediated diseases. Recent studies have demonstrated that the defect in the clearance of immune complexes and apoptotic cells is associated with autoimmune disease. Mice deficient in Clq show a lupus-like phenotype with the appearance of antinuclear antibodies and glomerulonephritis due to a defect in the clearance of immune complexes and apoptotic cells. This at least explains the paradox that, in humans, deficiency in an early complement component is a major risk factor for SLE. It is demonstrated that mutations in factor H, membrane cofactor protein (MCP) and factor I gene are associated with atypical hemolytic uremic syndrome. Since the complement system is a central mediator of inflammation, it is recognized as a promising therapeutic target. Anti-C5 monoclonal antibody was developed to block the final stage of complement activation. Pexelizumab is a single chain, short-acting anti-C5 antibody and is used for reperfusion after myocardial infarction, or for coronary artery bypass graft surgery with cardiopulmonary bypass. Eculizumab is a long-acting anti-C5 antibody used for paroxysmal nocturnal hemoglobinuria, rheumatoid arthritis, membranous glomerulonephritis with promising results.

Original languageEnglish
Pages (from-to)757-762
Number of pages6
JournalRinsho byori. The Japanese journal of clinical pathology
Volume54
Issue number7
Publication statusPublished - Jan 1 2006

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Immune System Diseases
Antigen-Antibody Complex
Anti-Idiotypic Antibodies
CD46 Antigens
Paroxysmal Hemoglobinuria
Complement Factor H
Inflammation Mediators
Membranous Glomerulonephritis
Complement Activation
Antinuclear Antibodies
Glomerulonephritis
Cardiopulmonary Bypass
Lectins
Innate Immunity
Coronary Artery Bypass
Autoimmune Diseases
Reperfusion
Rheumatoid Arthritis
Myocardial Infarction
Monoclonal Antibodies

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

Clinical aspects of the complement system. / Tsukamoto, Hiroshi; Horiuchi, Takahiko.

In: Rinsho byori. The Japanese journal of clinical pathology, Vol. 54, No. 7, 01.01.2006, p. 757-762.

Research output: Contribution to journalReview article

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