Complement Factor H: Physiology and Pathophysiology

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