Duncan Renee C, Wijeyewickrema Lakshmi C, Pike Robert N
Department of Biochemistry & Molecular Biology, Monash University, Clayton, Victoria 3800, Australia.
Biochimie. 2008 Feb;90(2):387-95. doi: 10.1016/j.biochi.2007.07.023. Epub 2007 Aug 6.
The complement system is a vital component of the host immune system, but when dysregulated, can also cause disease. The system is activated by three pathways: classical, lectin and alternative. The initiating proteases of the classical and lectin pathways have similar domain structure and employ similar mechanisms of activation. The C1r, C1s and MASP-2 proteases have the most defined roles in the activation of the system. This review focuses on the mechanisms whereby their interaction with substrates and inhibitors is regulated.
补体系统是宿主免疫系统的重要组成部分，但当调节失调时，也会引发疾病。该系统通过三条途径激活：经典途径、凝集素途径和替代途径。经典途径和凝集素途径的起始蛋白酶具有相似的结构域结构，并采用相似的激活机制。C1r、C1s和MASP-2蛋白酶在系统激活中具有最明确的作用。本综述重点关注它们与底物和抑制剂相互作用的调节机制。
