Human CFHR1 ELISA Kit

Supplementary info

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

Activity summary

CFHR1 also known as Complement Factor H-Related protein 1 plays a role in the regulation of the complement system. This protein has a molecular mass of approximately 37 kDa. CFHR1 is mainly expressed in the liver and is present in plasma. The protein has structural similarities to Factor H with homologous domains known as short consensus repeats which contribute to its function in modulating complement activity.

Biological function summary

CFHR1 regulates the alternative pathway of the complement system. It can bind to complement component C3b inhibiting its activity and preventing over-activation of the complement cascade. CFHR1 does not function alone; it interacts with other proteins in the complement system although it forms no stable complex. Through these interactions CFHR1 maintains homeostasis in immune responses.

Pathways

CFHR1 participates significantly in the alternative complement pathway. It works alongside other complement factors such as Factor H to ensure balanced complement activation and regulation. CFHR1 indirectly influences the inflammatory response by regulating this pathway linking innate immunity processes with complement-mediated functions.

Associated diseases and disorders

CFHR1 is linked to atypical hemolytic uremic syndrome (aHUS) and age-related macular degeneration (AMD). In aHUS CFHR1 gene deletions can lead to unrestrained complement activation due to lack of regulation which involves Factor H. Similarly in AMD altered CFHR1 expression can affect complement regulation in the retinal tissue involving interactions with C3b and Factor H.