A serum glycoprotein (CFH, β-1H globulin, 1231 aa) that regulates the alternate complement pathway by binding to C3b, accelerating the decay of the alternate pathway convertase C3bBb, and acting as a cofactor for complement factor I. Genetic variations in CFH are associated with basal laminar drusen and with age-related macular degeneration type 4. Deficiency of CFH allows uncontrolled activation of the alternate pathway and is associated with a number of renal diseases, including membranoproliferative glomerulonephritis and atypical haemolytic uraemic syndrome. There is also increased susceptibility to meningococcal infections.
Subjects: Medicine and Health.