Article
Mutations in components of complement influence the outcome of Factor I-associated atypical hemolytic uremic syndrome.
Kidney international - 1 Feb 2010
Bienaime Frank, Dragon-Durey Marie-Agnes, Regnier Catherine H, Nilsson Sara C, Kwan Wing H, Blouin Jacques, Jablonski Mathieu, Renault Nicolas, Rameix-Welti Marie-Anne, Loirat Chantal, Sautés-Fridman Catherine, Villoutreix Bruno O, Blom Anna M, Fremeaux-Bacchi Veronique
Abstract excerpt
Genetic studies have shown that mutations of complement inhibitors such as membrane cofactor protein, Factors H, I, or B and C3 predispose patients to atypical hemolytic uremic syndrome (aHUS). Factor I is a circulating serine protease that inhibits complement by degrading C3b and up to now only...
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