The Factor H gene family is a multidomain, multifunctional protein family whose individual members are defined by conserved structural elements, which display diverse yet often overlapping functions. These proteins share a common structural motif, the short consensus repeat (SCR), which is structurally conserved among related genes and between phylogenetically divergent species. The human complement Factor H (FH, CFH, HUS, b-1H) gene encodes a 1213 amino acid serum glycoprotein which is arranged into 20 SCRs, each approximately 60 amino acids long, and an 18-residue leader sequence. Factor H controls the function of the alternative complement pathway and acts as a cofactor with Factor I (C3b inactivator). In addition, Factor H has functional activity outside of the complement system, where it can bind to the cellular integrin receptor (CD11b/CD18), interact with cell surface glycosaminoglycans and associate with the surface of certain pathogenic microorganisms. Deficiencies in Factor H is a common characteristic of acute renal disease.
