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

Santiago Rodríguez de Córdoba; Jorge Esparza-Gordillo; Elena Goicoechea de Jorge; Margarita Lopez-Trascasa; Pilar Sánchez-Corral

doi:10.1016/j.molimm.2004.02.005

PubMed · 15163532

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

Santiago Rodríguez de Córdoba·Jorge Esparza-Gordillo·Elena Goicoechea de Jorge·Margarita Lopez-Trascasa·Pilar Sánchez-Corral

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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.

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Bacteria Chromosomes, Human, Pair 1 Complement Activation Complement Factor H Genes Genetic Variation Glomerulonephritis Hemolytic-Uremic Syndrome Homeostasis Humans Mutation Neoplasms Polymorphism, Genetic Protein Structure, Tertiary Structure-Activity Relationship Journal Article Research Support, Non-U.S. Gov't Review

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Santiago Rodríguez de Córdoba, Jorge Esparza-Gordillo, Elena Goicoechea de Jorge, Margarita Lopez-Trascasa, Pilar Sánchez-Corral. 2004. The human complement factor H: functional roles, genetic variations and disease associations.. https://doi.org/10.1016/j.molimm.2004.02.005

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