补体替代途径中相关因子的激活与年龄相关性黄斑变性的关系

Relationship between some relative factors of the complement alternative pathway and age-related macular degeneration

关键词:  年龄相关性黄斑变性  C3  H因子  H因子相关蛋白1和3  B因子  I因子

Key words:  [Key words]　age related macular degeneration  complement component 3(C3)  complement factor H(CFH）  complement factor H-related 1 and 3(CFHR1 and CFHR3)  complement factor B (CFB)  complement factor I(CFI）

摘要:

[摘要]　年龄相关性黄斑变性（age related macular dengeneration, AMD）是一种与年龄相关的黄斑区退行性病变，最终可导致视力模糊甚至丧失。AMD的发生受到环境和体内炎症反应综合影响，但其具体发病机制至今仍不清楚，目前研究发现由补体替代途径所介导的炎症反应可能起到关键作用。C3 (complement component 3)、H因子（complement factor H,CFH）、H因子相关蛋白1和3（complement factor H-related 1 and 3，CFHR1 and CFHR3)、B因子（complement factor B，CFB)、I因子（complement factor I，CFI）在替代途径的激活过程中起到了重要的调节作用。CFH可协同由CFI介导的C3b的裂解过程从而抑制C3转化酶形成，阻滞替代途径的激活。CFHR1和CFHR3作为CFH竞争性因子与CFH争夺C3b上的靶位点并可与CFI协同作用影响补体替代途径的激活。CFB和C3是补体替代途径中的关键因子与AMD中补体替代途径的激活有着密不可分的关系。本文就上述因子的激活在AMD发病过程中的作用进行综述。

Abstract:

[Abstract]　Age related macular degeneration (AMD) is a degenerative disease with the pathological changes in macula lutea and finally leads to the blurred vision even blindness. Environmental and inflammatory reaction may be related with its development. However the exact etiology of the diseases is not clear. AMD is likely a local response of complement alternative pathway which responds to certain systemic inflammatory diseases. Complement component 3 (C3), complement factor H (CFH), complement factor H-related 1 and 3 (CFHR1 and CFHR3), complement factor B (CFB), and complement factor I (CFI) are important components in the complement system and also the keys to the pathogenesis of AMD. This review is aiming to clarify possible functions of these factors based on recent research.