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CP Classical pathway

By David Kavanagh, Tim Goodship and D. KavanaghT. Goodship and D. Kavanagh

Abstract

Abstract Central to the pathogenesis of atypical hemolytic uremic syndrome (aHUS) is over-activation of the alternative pathway of complement. Following the initial discovery of mutations in the complement regulatory protein, factor H, mutations have been described in factor I, membrane cofactor protein and thrombomodulin, which also result in decreased complement regulation. Autoantibodies to factor H have also been reported to impair complement regulation in aHUS. More recently, gain of function mutations in the complement components C3 and Factor B have been seen. This review focuses on the genetic causes of aHUS, their functional consequences, and clinical effect

Topics: CCPs Complement control protein modules
Year: 2013
OAI identifier: oai:CiteSeerX.psu:10.1.1.351.9548
Provided by: CiteSeerX
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