Article
Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation.
Blood - 1 Jun 2008
Ståhl Anne-lie, Vaziri-Sani Fariba, Heinen Stefan, Kristoffersson Ann-Charlotte, Gydell Karl-Henrik, Raafat Reem, Gutierrez Alberto, Beringer Ortraud, Zipfel Peter F, Karpman Diana
Abstract excerpt
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...
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