Factor H-related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation.

@article{Heinen2009FactorHP,
  title={Factor H-related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation.},
  author={Stefan Heinen and Andrea Hartmann and Nadine Lauer and Ulrike Wiehl and H. M. Dahse and Sylvia Schirmer and Katharina Gropp and Tina Enghardt and Reinhard Wallich and Steffi Haelbich and Michael Mihlan and Ursula M. Schl{\"o}tzer-Schrehardt and Peter F Zipfel and Christine Skerka},
  journal={Blood},
  year={2009},
  volume={114 12},
  pages={2439-47}
}
Homozygous deletion of a 84-kb genomic fragment in human chromosome 1 that encompasses the CFHR1 and CFHR3 genes represents a risk factor for hemolytic uremic syndrome (HUS) but has a protective effect in age-related macular degeneration (AMD). Here we identify CFHR1 as a novel inhibitor of the complement pathway that blocks C5 convertase activity and interferes with C5b surface deposition and MAC formation. This activity is distinct from complement factor H, and apparently factor H and CFHR1… CONTINUE READING