Factor H-related proteins.
Factor H-related proteins (CFHRs) are plasma glycoproteins related in structure and antigenicity to each other and to the complement inhibitory protein factor H. Such proteins are found in most mammals but their number and domain composition vary. This chapter summarizes our current knowledge on the human factor H-related proteins. In contrast to factor H, they have no strong complement inhibitory activity, although for some of them regulatory or complement modulatory activity has been reported. A common feature of CFHRs is that they bind to the C3b component of complement. Novel links between CFHRs and various diseases (C3 glomerulopathies, atypical hemolytic uremic syndrome and age-related macular degeneration) have been revealed in recent years, but we are still far from understanding their biological function.
Junior Research Group Cellular Immunobiology, Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany.
Mihály
M
Józsi
Seppo
S
Meri
D001053:Apolipoproteins / Q000235:genetics / Q000378:metabolism; D001798:Blood Proteins / Q000235:genetics / Q000378:metabolism; D017242:Complement Factor H / Q000235:genetics / Q000378:metabolism*; D003165:Complement System Proteins / Q000235:genetics / Q000378:metabolism; D018628:Gene Dosage; D006801:Humans; D008024:Ligands; D005810:Multigene Family; D020033:Protein Isoforms
D001053:Apolipoproteins; D001798:Blood Proteins; C105970:CFHR3 protein, human; C105092:CFHR4 protein, human; C432223:CFHR5 protein, human; D008024:Ligands; D020033:Protein Isoforms; C100631:factor H-related protein 1; C100632:factor H-related protein 2; D017242:Complement Factor H; D003165:Complement System Proteins