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补体因子H家族蛋白与人类疾病。

Factor H family proteins and human diseases.

作者信息

Józsi Mihály, Zipfel Peter F

机构信息

Junior Research Group Cellular Immunobiology, Leibniz Institute for Natural Product Research and Infection Biology-Hans Knöll Institute, Jena D-07745, Germany.

出版信息

Trends Immunol. 2008 Aug;29(8):380-7. doi: 10.1016/j.it.2008.04.008. Epub 2008 Jul 2.

Abstract

Complement is a major defense system of innate immunity and aimed to destroy microbes. One of the central complement regulators is factor H, which belongs to a protein family that includes CFHL1 and five factor H-related (CFHR) proteins. Recent evidence shows that factor H family proteins (factor H and CFHRs) are associated with diverse and severe human diseases and are also used by human pathogenic microbes for complement evasion. Therefore, dissecting the exact functions of the individual CFHR proteins will provide insights into the pathophysiology of such inflammatory and infectious diseases and will define the therapeutic potential of these proteins.

摘要

补体是先天性免疫的主要防御系统,旨在破坏微生物。补体的核心调节因子之一是H因子,它属于一个蛋白质家族,该家族包括CFHL1和五种H因子相关(CFHR)蛋白。最近的证据表明,H因子家族蛋白(H因子和CFHRs)与多种严重的人类疾病相关,并且人类致病微生物也利用它们来逃避补体。因此,剖析各个CFHR蛋白的确切功能将有助于深入了解此类炎症和感染性疾病的病理生理学,并确定这些蛋白的治疗潜力。

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