Semin Thromb Hemost 2006; 32(2): 146-154
DOI: 10.1055/s-2006-939770
Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

The Role of Defective Complement Control in Hemolytic Uremic Syndrome

Peter F. Zipfel1 , 2 , Joachim Misselwitz3 , Christoph Licht4 , Christine Skerka1
  • 1Department of Infection Biology, Leibniz-Institute for Natural Product Research, Jena, Germany
  • 2Infection Biology; Hans-Knoell-Institute, Jena, Germany
  • 3Department of Pediatric Nephrology, Friedrich-Schiller-University, Jena, Germany
  • 4Children's Hospital of the University of Cologne, Pediatric Nephrology, Cologne, Germany
Further Information

Publication History

Publication Date:
30 March 2006 (online)

ABSTRACT

Atypical hemolytic uremic syndrome (HUS) is a severe disease that is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Recent evidence has shown that defective complement activation and defective complement control is a cause of HUS. So far, mutations in single genes coding for the cofactor and complement regulator factor H, the membrane cofactor protein (MCP/CD46), the serine protease factor I, and autoantibodies to factor H have been linked to HUS. All of these proteins affect the same enzyme the alternative pathway convertase C3bBb. This article explains how alternative pathway activation proceeds and how defective control increases activation, which ultimately leads to endothelial cell damage.

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Peter F ZipfelPh.D. 

Professor, Department of Infection Biology, Leibniz-Institute for Natural Product Research and Infection Biology, Beutenbergstr

11a, 07745 Jena, Germany

Email: peter.zipfel@hki-jena.de

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