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The human complement factor H: functional roles, genetic variations and disease associations

2004; Elsevier BV; Volume: 41; Issue: 4 Linguagem: Inglês

10.1016/j.molimm.2004.02.005

1872-9142

S RODRIGUEZDECORDOBA, Jorge Esparza-Gordillo, Elena Goicoechea de Jorge, Margarita López‐Trascasa, Pilar Sánchez‐Corral,

Erythrocyte Function and Pathophysiology

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.