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Semin Thromb Hemost 2001; 27(3): 191-200
DOI: 10.1055/s-2001-15248
Copyright © 2001 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Complement Factor H: Physiology and Pathophysiology

Peter F. Zipfel
  • Department of Infectious Biology, Hans-Knoell Institute for Natural Products Research, Jena, Germany
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Publication History

Publication Date:
31 December 2001 (online)

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ABSTRACT

The human plasma protein factor H, which is a multifunctional, multidomain protein, acts as a central regulator of the complement system. In addition to its complement regulatory activities, factor H has multiple physiological activities and 1) acts as an extracellular matrix component, 2) binds to cellular receptors of the integrin type, and 3) interacts with a wide selection of ligands, such as the C-reactive protein, thrombospondin, bone sialoprotein, osteopontin, and heparin. Recent genetic reports, which show involvement of factor H in the human disease hemolytic-uremic syndrome (HUS), have attracted the attention of both clinicians and basic complement researchers to the role of factor H in the pathophysiology of HUS.

KEYWORD

Hemolytic-uremic syndrome - HUS - complement system - complement factor H - physiological functions of factor H

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