Recombinant Mouse Protein beta-1-H/CFH(C-6His)

Source : Human Cells,Complement factor H(CFH) is a 155 kDa glycoprotein that functions as a cofactor in the inactivation of C3b by factor I. It also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway. CFH expressed by the liver and secreted in plasma. This recombinant protein corresponds to SCR15-20 which encompass the primary binding sites for heparin and C3b as well as for the peptide hormone adrenomedullin. Within SCR15-20, mouse Factor H shares 60% and 80% amino acid sequence identity with human and rat Factor H, respectively. Dozens of mutations clustered in SCR15-20 are associated with atypical hemolytic uremic syndrome, a disorder characterized by anemia, thrombocytopenia, and renal failure. Protein function: Glycoprotein that plays an essential role in maintaining a well-balanced immune response by modulating complement activation. Acts as a soluble inhibitor of complement, where its binding to self markers such as glycan structures prevents complement activation and amplification on cell surfaces. Accelerates the decay of the complement alternative pathway (AP) C3 convertase C3bBb, thus preventing local formation of more C3b, the central player of the complement amplification loop. As a cofactor of the serine protease factor I, CFH also regulates proteolytic degradation of already-deposited C3b. In addition, mediates several cellular responses through interaction with specific receptors. For example, interacts with CR3/ITGAM receptor and thereby mediates the adhesion of human neutrophils to different pathogens. In turn, these pathogens are phagocytosed and destroyed. [The UniProt Consortium]