CFB Human, Native
Human Plasma.
CFB, C3/C5 convertase, Glycine-rich beta glycoprotein, GBG, PBF2, Properdin factor B, BF, BFD, AHUS4, ARMD14, CFAB, CFBD, FB, FBI12, GBG, H2-Bf.
Sterile filtered solution.
Greater than 95.0% as determined by SDS-PAGE.
Description
Human Complement Factor B produced in Human plasma is glycosylated polypeptide chain having a total molecular mass of 93kDa.
Product Specs
Complement Factor B, also referred to as CFB, is a protein encoded by the CFB gene. This protein is a key element of the complement system's alternative pathway. CFB, circulating in the bloodstream as a single polypeptide chain, undergoes cleavage upon activation of the alternative pathway. This cleavage, facilitated by complement factor D, produces two fragments: Ba, a non-catalytic chain, and Bb, a catalytic subunit. The active Bb subunit, a serine protease, interacts with C3b to form the alternative pathway C3 convertase, a crucial enzyme in the complement cascade. Furthermore, Bb plays a role in the proliferation of preactivated B lymphocytes, while Ba, conversely, inhibits their proliferation.
Human Complement Factor B, derived from human plasma, is a glycosylated polypeptide chain with a molecular weight of 93 kDa.
A sterile solution that has been filtered.
The CFB protein solution is buffered with 10mM Sodium phosphate and 145mM NaCl, at a pH of 7.2.
For optimal stability, CFB Human should be stored at 4°C and used within 2-4 weeks of opening the vial. For extended storage, freezing below -20°C is recommended. The addition of a carrier protein (0.1% HSA or BSA) is advised for long-term storage. Repeated freezing and thawing should be avoided.
Purity exceeds 95.0% as assessed by SDS-PAGE analysis.
Each plasma donation has undergone rigorous testing and is confirmed negative for antibodies against HIV-1, HIV-2, HCV, and the surface antigen of the Hepatitis B virus (HBsAg).
CFB, C3/C5 convertase, Glycine-rich beta glycoprotein, GBG, PBF2, Properdin factor B, BF, BFD, AHUS4, ARMD14, CFAB, CFBD, FB, FBI12, GBG, H2-Bf.
Human Plasma.
Product Science Overview
The CFB gene is located on chromosome 6 in humans . The gene encodes a single-chain polypeptide that circulates in the blood. Upon activation, Complement Factor B is cleaved by Complement Factor D into two fragments: Ba and Bb . The Bb fragment is a serine protease that combines with Complement Factor 3b (C3b) to form the C3/C5 convertase, which is essential for the activation of the complement system .
Complement Factor B is integral to the alternative pathway of complement activation. This pathway is part of the body’s first line of defense against pathogens. When the alternative pathway is activated, Factor B is cleaved, and the Bb fragment associates with C3b to form the C3 convertase (C3bBb). This complex amplifies the response by cleaving more C3 molecules, leading to the opsonization of pathogens and the formation of the membrane attack complex (MAC), which can lyse invading cells .
The Bb fragment has several important functions:
Mutations or deficiencies in Complement Factor B can lead to various immune disorders. For instance, Complement Factor B deficiency (CFBD) is characterized by an increased susceptibility to bacterial infections, particularly those caused by Neisseria species . Additionally, certain variants of the CFB gene have been associated with age-related macular degeneration (ARMD14), highlighting its role in ocular health .
The crystal structure of human Complement Factor B has been resolved at a 2.3-angstrom resolution, providing insights into how the five-domain proenzyme is kept inactive until needed . This structural information is crucial for understanding the precise mechanisms of complement activation and for developing therapeutic interventions.
