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Factor H and disease: a complement regulator affects vital body functions.

P F Zipfel1, J Hellwage, M A Friese

  • 1Research Group for Biomolecular Medicine, Bernhard Nocht Institute for Tropical Medicine, Hamburg, Germany. zipfel@uke.uni-hamburg.de

Molecular Immunology
|July 14, 1999
PubMed
Summary

Factor H is a crucial complement regulator. Its dysfunction is linked to severe diseases like glomerulonephritis and hemolytic uremic syndrome, and pathogens exploit it to evade immune responses.

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Area of Science:

  • Immunology
  • Molecular Biology
  • Genetics

Background:

  • Factor H is a multifunctional plasma protein regulating the complement system.
  • It controls C3b fate and C3 convertase formation on fluid and cell surfaces.
  • Factor H also exhibits non-complement functions, including adhesion and chemotaxis.

Purpose of the Study:

  • To summarize current knowledge on factor H's role in human diseases.
  • To highlight the association between factor H dysfunction and pathological conditions.
  • To review how pathogens interact with factor H for immune evasion.

Main Methods:

  • Review of genetic and pathophysiological analyses.
  • Summary of studies on factor H function and interactions.
  • Analysis of factor H's role in complement regulation and disease pathogenesis.

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Main Results:

  • Factor H deficiency or absence causes uncontrolled C3 consumption.
  • Reduced factor H levels or mutations are associated with glomerulonephritis and hemolytic uremic syndrome.
  • Microbes like Streptococcus pyogenes and Neisseria gonorrhoeae bind factor H to evade complement attack.

Conclusions:

  • Factor H is essential for maintaining complement homeostasis.
  • Abnormal factor H function is directly implicated in serious human diseases.
  • Understanding factor H's interactions is key to comprehending complement-related pathologies and microbial evasion strategies.