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

Mol Immunol. 2004 Jun;41(4):355-67. doi: 10.1016/j.molimm.2004.02.005.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Bacteria / immunology
  • Chromosomes, Human, Pair 1 / genetics
  • Complement Activation
  • Complement Factor H / chemistry
  • Complement Factor H / genetics
  • Complement Factor H / physiology*
  • Genes
  • Genetic Variation
  • Glomerulonephritis / genetics
  • Glomerulonephritis / immunology
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / immunology
  • Homeostasis
  • Humans
  • Mutation
  • Neoplasms / immunology
  • Polymorphism, Genetic
  • Protein Structure, Tertiary
  • Structure-Activity Relationship

Substances

  • CFH protein, human
  • Complement Factor H