Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation.

@article{Sthl2008FactorHD,
  title={Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation.},
  author={Anne-lie St{\aa}hl and Fariba Vaziri-Sani and Stefan Heinen and Ann-Charlotte Kristoffersson and Karl-Henrik Gydell and Reem H Raafat and Alberto Guti{\'e}rrez and Ortraud Beringer and Peter F Zipfel and Diana Karpman},
  journal={Blood},
  year={2008},
  volume={111 11},
  pages={5307-15}
}
Atypical hemolytic uremic syndrome (aHUS) may be associated with mutations in the C-terminal of factor H (FH). FH binds to platelets via the C-terminal as previously shown using a construct consisting of short consensus repeats (SCRs) 15 to 20. A total of 4 FH mutations, in SCR15 (C870R) and SCR20 (V1168E, E1198K, and E1198Stop) in patients with aHUS, were studied regarding their ability to allow complement activation on platelet surfaces. Purified FH-E1198Stop mutant exhibited reduced binding… CONTINUE READING

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