Complement factor H-related protein 4 (CFHR4)

Complement factor H-related protein 4 (CFHR4) is a member of the CFH family, comprising five closely linked genes encoding secreted plasma proteins primarily synthesized in the liver. CFHR4 contains several short consensus repeats (SCRs) and modulates complement activation by binding C3b and other ligands, thus competing with major complement regulators (factor H and FHL-1) for binding sites. In the eye, accumulation of FHR-4 is associated with increased complement activation and inflammation, implicated especially in the pathogenesis of age-related macular degeneration. Genetic variants of CFHR4 can alter systemic and local concentrations, influencing disease risk. The protein may also be involved in lipid metabolism and immune response, but its functions are primarily related to complement regulation and modulation.

Other names

CFHL4FHR4FHR-4complement factor H-related protein 4CFHR4

Competes with complement factor H and FHL-1 for binding C3b, altering complement regulation. Facilitates complement activation and inflammation when dysregulated.