Anti-CFHR4 antibody [EPR25760-27] - BSA and Azide free (Detector)

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

CFHR4 also known as Complement Factor H-Related 4 is a protein involved in the regulation of the complement system which plays an essential role in innate immunity. CFHR4 has a mass of approximately 86 kDa. The protein is part of the complement factor H protein family and expresses primarily in the liver and plasma. The protein features several short consensus repeat (SCR) domains that facilitate its interaction within the complement pathway therefore influencing immune system activity.

Biological function summary

CFHR4 modulates the activity of the complement system by binding to C3b a central component in complement activation. It is not a direct inhibitor but competes with Complement Factor H (CFH) for binding sites influencing factor H-mediated regulation. This positioning helps maintain a balance in complement activation and inhibition possibly preventing overactivation which could damage host tissues. CFHR4 does not form large complexes but operates closely with other complement regulators like CFH.

Pathways

CFHR4 participates in the alternative complement pathway. The alternative pathway acts as a continuous surveillance system that does not require antibody-antigen interactions for activation. CFHR4 interacts with complement components such as C3b and CFH within this pathway contributing to the control and fine-tuning of complement activity under normal physiological conditions. CFH and CFHR4 both play a role ensuring that the complement system responds accurately to threats without damaging host cells.

Associated diseases and disorders

CFHR4 has implications in diseases like age-related macular degeneration (AMD) and atypical hemolytic uremic syndrome (aHUS). Variants or dysregulation of CFHR4 may disrupt complement control leading to inflammation and tissue damage associated with these conditions. The relationship between CFHR4 CFH and other complement components like C3b reveals the importance of balanced complement control in preventing these diseases. Understanding CFHR4's role assists researchers and clinicians in developing therapeutic strategies for conditions related to complement dysregulation.