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补体系统在健康与疾病中的作用

Complement in health and disease.

机构信息

Department of Pharmacology, University of Oxford, Mansfield Road, Oxford OX1 3QT, UK.

出版信息

Adv Drug Deliv Rev. 2011 Sep 16;63(12):965-75. doi: 10.1016/j.addr.2011.06.005. Epub 2011 Jun 16.


DOI:10.1016/j.addr.2011.06.005
PMID:21704094
Abstract

The complement system consists of about 35-40 proteins and glycoproteins present in blood plasma or on cell surfaces. Its main biological function is to recognise "foreign" particles and macromolecules, and to promote their elimination either by opsonisation or lysis. Although historically complement has been studied as a system for immune defence against bacteria, it has an important homeostatic role in which it recognises damaged or altered "self" components. Thus complement has major roles in both immune defence against microorganisms, and in clearance of damaged or "used" host components. Since complement proteins opsonise or lyse cells, complement can damage healthy host cells and tissues. The system is regulated by many endogenous regulatory proteins. Regulation is sometimes imperfect and both too much and too little complement activation is associated with many diseases. Excessive or inappropriate activation can cause tissue damage in diseases such as rheumatoid arthritis, age-related macular degeneration (AMD), multiple sclerosis, ischemia-reperfusion injury (e.g. ischemic stroke). Insufficient complement activity is associated with susceptibility to infection (mainly bacterial) and development of autoimmune disease, like SLE (systemic lupus erythematosus).

摘要

补体系统由约 35-40 种存在于血浆或细胞表面的蛋白质和糖蛋白组成。其主要生物学功能是识别“外来”颗粒和大分子,并通过调理作用或溶解作用促进其清除。尽管补体在历史上一直被作为针对细菌的免疫防御系统进行研究,但它在识别受损或改变的“自身”成分方面具有重要的稳态作用。因此,补体在微生物免疫防御和清除受损或“使用”的宿主成分方面都具有重要作用。由于补体蛋白调理或溶解细胞,补体可能会损伤健康的宿主细胞和组织。该系统受许多内源性调节蛋白的调节。调节有时并不完美,补体激活过多或过少都与许多疾病有关。过度或不适当的激活可导致类风湿关节炎、年龄相关性黄斑变性 (AMD)、多发性硬化症、缺血再灌注损伤(如缺血性中风)等疾病中的组织损伤。补体活性不足与易感染(主要是细菌)和自身免疫性疾病(如系统性红斑狼疮 (SLE))的发生有关。

相似文献

[1]
Complement in health and disease.

Adv Drug Deliv Rev. 2011-6-16

[2]
Inherited complement regulatory protein deficiency predisposes to human disease in acute injury and chronic inflammatory statesthe examples of vascular damage in atypical hemolytic uremic syndrome and debris accumulation in age-related macular degeneration.

Adv Immunol. 2007

[3]
Role of complement in innate and autoimmunity.

J Nephrol. 2005

[4]
Deficiencies and excessive human complement system activation in disorders of multifarious etiology.

Adv Clin Exp Med. 2012

[5]
Mechanisms of Disease: the complement system and the pathogenesis of systemic lupus erythematosus.

Nat Clin Pract Rheumatol. 2006-6

[6]
[Exploration of the complement system in clinical practice].

Arch Pediatr. 1994-1

[7]
[The role of complement system in infections].

Tidsskr Nor Laegeforen. 1991-1-10

[8]
Complement activation and inhibition: a delicate balance.

Trends Immunol. 2009-2

[9]
The complement system: pathophysiology and clinical relevance.

Wien Klin Wochenschr. 1999-5-21

[10]
Immune complex modulation by plasma proteins. With special reference to the complement system and autoimmune diseases.

Dan Med Bull. 1989-10

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