CFH Human
Human Plasma.
Complement factor H, H factor 1, CFH, HF, HF1, HF2.
Sterile filtered solution.
Greater than 95.0% as determined by SDS-PAGE.
Description
Human Complement Factor H produced in Human plasma having a total molecular mass of 155kDa.
Product Specs
A key regulator of the alternative complement pathway is complement factor H (CFH). CFH is essential for preventing complement activation on host cells and tissues, particularly in the kidney. By acting as a cofactor for factor I, which proteolytically inactivates C3b when it is bound to CFH, CFH regulates the formation and breakdown of the alternative pathway C3/C5 convertase. The N-terminal 5 domains of CFH bind to C3b and block factor B binding, which lowers the synthesis of C3/C5 convertase. Preformed C3/C5 convertases also bind to CFH, which results in the catalytic subunit Bb being quickly released. These actions are necessary to regulate how the alternative pathway amplification process spontaneously activates in plasma. Additionally, CFH regulates the formation and breakdown of these enzymes when C3b is bound to the surface of particles.
Produced in human plasma, Human Complement Factor H has a molecular weight of 155kDa.
A sterile-filtered solution.
The CFH protein solution is prepared with PBS at a pH of 7.2.
If the entire vial will be used within 2-4 weeks, CFH Human is stable at 4°C. For longer storage periods, keep frozen below -20°C. It is recommended to add a carrier protein (0.1% HSA or BSA) for long-term storage. It is important to avoid repeated freeze-thaw cycles.
When tested using SDS-PAGE, the purity is greater than 95.0%.
Plasma from all donors has been tested and found to be negative for antibodies to HIV-1, HIV-2, HCV, and HBSAG.
Complement factor H, H factor 1, CFH, HF, HF1, HF2.
Human Plasma.
Product Science Overview
Complement Factor H is a large glycoprotein with a molecular weight of approximately 155 kilodaltons. It is composed of 20 complement control protein (CCP) modules, also known as short consensus repeats or sushi domains. These modules are connected by short linkers and arranged in an extended head-to-tail fashion .
The primary function of CFH is to regulate the alternative pathway of the complement system. It achieves this by binding to self markers such as glycan structures on host cells, preventing complement activation and amplification on these surfaces. This regulation is crucial for protecting host tissues from damage caused by the complement system .
CFH exerts its regulatory effects through several mechanisms:
- Decay-Accelerating Activity: CFH accelerates the decay of the complement alternative pathway C3 convertase (C3bBb), preventing the formation of more C3b, which is central to the complement amplification loop .
- Cofactor Activity: CFH acts as a cofactor for the serine protease Factor I, facilitating the proteolytic degradation of already-deposited C3b .
- Interaction with Pathogens: CFH can bind to specific receptors on pathogens, mediating their phagocytosis and destruction by human neutrophils .
CFH is essential for preventing complement activation on host cells and tissues, particularly in the kidneys. Mutations or deficiencies in CFH can lead to various diseases, including atypical hemolytic uremic syndrome (aHUS), age-related macular degeneration (AMD), and membranoproliferative glomerulonephritis (MPGN) .
