Complement factor H: physiology and pathophysiology

Semin Thromb Hemost. 2001 Jun;27(3):191-9. doi: 10.1055/s-2001-15248.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Complement Factor H / chemistry
  • Complement Factor H / immunology
  • Complement Factor H / physiology*
  • Glomerulonephritis, Membranoproliferative / etiology
  • Hemolytic-Uremic Syndrome / etiology
  • Hemolytic-Uremic Syndrome / physiopathology
  • Humans
  • Neoplasms / immunology


  • CFH protein, human
  • Complement Factor H