Complement factor H: physiology and pathophysiology.
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.
Zipfel
P F
PF
Department of Infectious Biology, Hans-Knoell Institute for Natural Products Research, Jena, Germany. zipfel@pmail.hki-jena.de
D017242:Complement Factor H / Q000737:chemistry / Q000276:immunology / Q000502:physiology*; D015432:Glomerulonephritis, Membranoproliferative / Q000209:etiology; D006463:Hemolytic-Uremic Syndrome / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D009369:Neoplasms / Q000276:immunology
C493495:CFH protein, human; D017242:Complement Factor H