Complement Factor H-related 4/CFHR4 Products

Description

Complement factor H-related protein 4 (CFHR4) is a secreted glycoprotein member of the factor H family of glycoproteins (1). The human complement factor H protein family consists of the complement and immune regulators factor H, the factor H-like protein 1 (FHL-1) and five factor H-related proteins (FHR-1 to -5) (1). The genes of this family are located on human chromosome 1q32, which is known as the regulator of complement activation (RCA) gene clusters (2). CFHRs are exclusively composed of individually folded protein domains, termed short consensus repeats (SCRs) or complement control modules. All CFHRs, including CFHR4, are capable of binding complement factor C3b, discriminate between self and non-self cell surfaces, and have been proposed to deregulate complement activation by inhibiting interaction of CFH with C3b and C3d (1, 3). CFHR4 activates complement through its interaction with C3b (4). CFHR4 can also bind to native (pentameric) C-reactive protein (pCRP) through its first SCR domain and is suggested to promote pCRP binding to necrotic cells surfaces, thus promoting cell clearance (5).  CFHR4 is synthesized as 2 isoforms by hepatocytes and circulates in plasma. The shorter isoform, FHR4B, contains 5 of 9 SCRs. The CFHR4 product includes a duplication insertion of 247 aa and represents the longer alternative splice form termed FHR4A (6). 

Bioinformatics

Uniprot	Q92496.3
Product By Gene ID	10877
Alternate Names	complement factor H-related protein 4 CFHL4 CFHR4 Complement Factor Hrelated 4 Complement Factor H-related 4 FHR4 FHR-4