title: The human complement factor H: functional roles, genetic variations and disease associations.
abstract: 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.
authors.affiliation: Centro de Investigaciones Biológicas, Departmento de Inmunologia, Ramiro de Maeztu 9, 28040 Madrid, Spain. srdecordoba@cib.csic.es
authors.forename: Santiago
authors.initials: S
authors.lastname: Rodríguez de Córdoba
authors.forename: Jorge
authors.initials: J
authors.lastname: Esparza-Gordillo
authors.forename: Elena
authors.initials: E
authors.lastname: Goicoechea de Jorge
authors.forename: Margarita
authors.initials: M
authors.lastname: Lopez-Trascasa
authors.forename: Pilar
authors.initials: P
authors.lastname: Sánchez-Corral
mesh_terms: D001419:Bacteria / Q000276:immunology; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics; D003167:Complement Activation; D017242:Complement Factor H / Q000737:chemistry / Q000235:genetics / Q000502:physiology*; D005796:Genes; D014644:Genetic Variation; D005921:Glomerulonephritis / Q000235:genetics / Q000276:immunology; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000276:immunology; D006706:Homeostasis; D006801:Humans; D009154:Mutation; D009369:Neoplasms / Q000276:immunology; D011110:Polymorphism, Genetic; D017434:Protein Structure, Tertiary; D013329:Structure-Activity Relationship
chemical_list: C493495:CFH protein, human; D017242:Complement Factor H