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Semin Thromb Hemost 2001; 27(3): 191-200
DOI: 10.1055/s-2001-15248
DOI: 10.1055/s-2001-15248
Complement Factor H: Physiology and Pathophysiology
Further Information
Publication History
Publication Date:
31 December 2001 (online)
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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