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Semin Thromb Hemost. 2001 Jun;27(3):191-9.

Complement factor H: physiology and pathophysiology.

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  • 1Department of Infectious Biology, Hans-Knoell Institute for Natural Products Research, Jena, Germany. zipfel@pmail.hki-jena.de

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.

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