Complement in human diseases: Lessons from complement deficiencies

被引：192

作者：

Botto, Marina ^{[2
]}

Kirschfink, Michael ^{[3
]}

Macor, Paolo ^{[1
]}

Pickering, Matthew C. ^{[2
]}

Wuerzner, Reinhard ^{[4
]}

Tedesco, Francesco ^{[1
]}

机构：

[1] Univ Trieste, Dept Life Sci, Trieste, Italy

[2] Univ London Imperial Coll Sci Technol & Med, Fac Med, Rheumatol Sect, London, England

[3] Univ Heidelberg, Inst Immunol, D-6900 Heidelberg, Germany

[4] Innsbruck Med Univ, Div Hyg & Med Microbiol, Innsbruck, Austria

来源：

MOLECULAR IMMUNOLOGY | 2009年 / 46卷 / 14期

基金：

英国惠康基金;

关键词：

Complement; Immunodeficiency; Bacterial infections; Immune-complex diseases; Diagnosis; Registry; SYSTEMIC-LUPUS-ERYTHEMATOSUS; MANNOSE-BINDING LECTIN; HEMOLYTIC-UREMIC SYNDROME; FACTOR-I DEFICIENCY; TRANSLATIONAL MINIREVIEW SERIES; MEMBRANE COFACTOR PROTEIN; FACTOR-H; MACULAR DEGENERATION; FACTOR-B; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS;

D O I：

10.1016/j.molimm.2009.04.029

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Complement deficient cases reported in the second half of the last century have been of great help in defining the role of complement in host defence. Surveys of the deficient individuals have been instrumental in the recognition of the clinical consequences of the deficiencies. This review focuses on the analysis of the diseases associated with the deficiencies of the various components and regulators of the complement system and their therapeutic implications. The diagnostic approach leading to the identification of the deficiency is discussed here as a multistep process that starts with the screening assays and proceeds in specialized laboratories with the characterization of the defect at the molecular level. The organization of a registry of complement deficiencies is presented as a means to collect the cases identified in and outside Europe with the aim to promote joint projects on treatment and prevention of diseases associated with defective complement function. (C) 2009 Elsevier Ltd. All rights reserved.

引用

页码：2774 / 2783

页数：10

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