Rodríguez de Córdoba Santiago, Esparza-Gordillo Jorge, Goicoechea de Jorge Elena, Lopez-Trascasa Margarita, Sánchez-Corral Pilar
Centro de Investigaciones Biológicas, Departmento de Inmunologia, Ramiro de Maeztu 9, 28040 Madrid, Spain.
Mol Immunol. 2004 Jun;41(4):355-67. doi: 10.1016/j.molimm.2004.02.005.
Factor H is an essential regulatory protein that plays a critical role in the homeostasis of the complement system in plasma and in the protection of bystander host cells and tissues from damage by complement activation. Genetic and structural data generated during recent years have been instrumental to delineate the functional domains responsible for these regulatory activities in factor H, which is helping to understand the molecular basis underlying the different pathologies associated to factor H. This review summarises our current knowledge of the role of factor H in health and disease.
补体因子H是一种重要的调节蛋白,在血浆补体系统的稳态维持以及保护旁观者宿主细胞和组织免受补体激活损伤方面发挥着关键作用。近年来产生的遗传和结构数据有助于确定补体因子H中负责这些调节活性的功能域,这有助于理解与补体因子H相关的不同病理学的分子基础。本综述总结了我们目前对补体因子H在健康和疾病中作用的认识。