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 Blood, 1 September 2007, Vol. 110, No. 5, pp. 1516-1518.
Prepublished online as a Blood First Edition Paper on May 10, 2007; DOI 10.1182/blood-2007-02-071472.

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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY

Brief Report

Anti–factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome

Mihály Józsi1, Stefanie Strobel1, Hans-Martin Dahse2, Wei-shih Liu3, Peter F. Hoyer3, Martin Oppermann4, Christine Skerka2, and Peter F. Zipfel2,5

1 Junior Research Group Cellular Immunobiology and 2 Department of Infection Biology, Leibniz Institute for Natural Product Research and Infection Biology, Hans Knöll Institute, Jena; 3 Department of Pediatric Nephrology, University Clinic Essen, University Duisburg-Essen, Essen; 4 Department of Cellular and Molecular Immunology, University of Göttingen, Göttingen; and 5 Friedrich Schiller University, Jena, Germany

The atypical form of the kidney disease hemolytic uremic syndrome (aHUS) is associated with defective complement regulation. In addition to mutations in complement regulators, factor H (FH)–specific autoantibodies have been reported for aHUS patients. The aim of the present study was to understand the role of these autoantibodies in aHUS. First, the binding sites of FH autoantibodies from 5 unrelated aHUS patients were mapped using recombinant FH fragments and competitor antibodies. For all 5 autoantibodies, the binding site was localized to the FH C-terminus. In a functional assay, isolated patient IgG inhibited FH binding to C3b. In addition, autoantibody-positive patients' plasma caused enhanced hemolysis of sheep erythrocytes, which was reversed by adding FH in excess. These results suggest that aHUS-associated FH autoantibodies mimic the effect of C-terminal FH mutations, as they inhibit the regulatory function of FH at cell surfaces by blocking its C-terminal recognition region.


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