The Role of Defective Complement Control in Hemolytic Uremic Syndrome

Christoph Licht; Christine Skerka; Joachim Misselwitz; Peter F. Zipfel

doi:10.1055/s-2006-939770

Seminars in Thrombosis and Hemostasis, Inhaltsverzeichnis

Semin Thromb Hemost 2006; 32(2): 146-154
DOI: 10.1055/s-2006-939770

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

Abstract

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.

KEYWORDS

Complement control - alternative pathway - hemolytic uremic syndrome (HUS) - membrane-proliferative glomerulonephritis (MPGN) - factor H - membrane cofactor protein (MCP) - factor I - autoantibodies

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Referenzen

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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