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DOI: 10.1055/s-2006-939770
The Role of Defective Complement Control in Hemolytic Uremic Syndrome
Publication History
Publication Date:
30 March 2006 (online)
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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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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