Complement factor B; C3/C5 convertase; Glycine-rich beta glycoprotein; GBG; PBF2; Properdin factor B; Complement factor B Ba fragment; Complement factor B Bb fragment; CFB; BF; BFD

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This BF antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 395-422 amino acids from the Central region of human BF.

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Vial Concentration: 2 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-CFB antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

BF is complement factor B, a component of the alternative pathway of complement activation. Factor B circulates in the blood as a single chain polypeptide. Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. The active subunit Bb is a serine protease which associates with C3b to form the alternative pathway C3 convertase. Bb is involved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation.

Immunology

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

Western blot analysis of BF Antibody (Center) in HepG2 cell line and mouse liver tissue lysates (35ug/lane). BF (arrow) was detected using the purified Pab.

BF Antibody (Center) (RB18685) IHC analysis in formalin fixed and paraffin embedded human hepatocarcinoma tissue followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the BF Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.

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complement factor B

complement factor B

Complement factor B

BF; BFD; GBG??[Similar Products]

CFAB_HUMAN

This gene encodes complement factor B, a component of the alternative pathway of complement activation. Factor B circulates in the blood as a single chain polypeptide. Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. The active subunit Bb is a serine protease which associates with C3b to form the alternative pathway C3 convertase. Bb is involved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. This cluster includes several genes involved in regulation of the immune reaction. Polymorphisms in this gene are associated with a reduced risk of age-related macular degeneration. The polyadenylation site of this gene is 421 bp from the 5' end of the gene for complement component 2. [provided by RefSeq, Jul 2008]

CFB: Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in proliferation and differentiation of preactivated B- lymphocytes, rapid spreading of peripheral blood monocytes, stimulation of lymphocyte blastogenesis and lysis of erythrocytes. Ba inhibits the proliferation of preactivated B-lymphocytes. Defects in CFB are a cause of susceptibility to hemolytic uremic syndrome atypical type 4 (AHUS4). An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Belongs to the peptidase S1 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Protease; EC 3.4.21.47; Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 6p21.3

Cellular Component: extracellular space; plasma membrane; extracellular region

Molecular Function: complement binding; serine-type endopeptidase activity

Biological Process: complement activation, alternative pathway; regulation of complement activation; innate immune response; proteolysis; complement activation

Disease: Macular Degeneration, Age-related, 14; Complement Factor B Deficiency; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 4

Baatz,H., Klin Monatsbl Augenheilkd 226 (8), 654-658 (2009)
Goring,K., Am. J. Physiol., Cell Physiol. 296 (5), C1140-C1150 (2009)

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