Description
Complement Factor H (CFH) is key in the complement system, vital for immune defense and inflammation control. With a robust presence in the bloodstream at 400-800 µg/ml, CFH stabilizes complement activity, targeting immune responses. It binds C3b, speeding up alternative pathway C3-convertase breakdown and aiding C3b deactivation, thereby shielding the body from undue tissue damage. CFH's structure, a 150 kDa glycoprotein with 20 consensus repeats, is crucial for its function. These repeats have sites essential for CFH's anti-inflammatory role and CRP interaction, affecting cell signaling. CFH's N-terminal and C-terminal domains, notably SCR 7, are key in fluid regulation and cellular defense. The link between CFH gene variations and a spectrum of human diseases, including kidney disorders like atypical Hemolytic Uremic Syndrome (aHUS) and vision-threatening conditions like age-related macular degeneration (AMD), underscore its clinical relevance and the potential for targeted CFH-based therapies.