Complement Factor B, Purified, (Polyclonal) (Rabbit IgG); Purified Rabbit Anti-Mouse Complement Factor B Polyclonal Antibody

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

Chromosome: 6; NC_000006.11 (31913721..31919861). Location: 6p21.3

Polyclonal

IgG

Rabbit

This antibody is specific for the mouse Complement Factor B/Bb. WB analysis shows that the antibody may also cross-react with the Ba fragment.

Store at + 4 degree C. For long term storage, aliquot and freeze unused portion at -20 degree C in volumes appropriate for single usage. Avoid 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.

Complement factor B is an essential protein in the alternative complement pathway. It is expressed by hepatocytes and macrophages and localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Factor B circulates in the blood as a single chain polypeptide of 764 amino acids (MW of 90 kDa). 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 that associates with C3b to form the alternative pathway C3 convertase. Bb is also involved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation. Factor B is an acute-phase protein whose levels increase during inflammation.

Western Blot (WB), ELISA (EIA)

Suggested minimum working dilution of 1:100 in Western Blot and 1:8000 in ELISA. This antibody has not been tested in other application.

complement factor B

complement factor B; C3 proactivator; C3/C5 convertase; C3 proaccelerator; properdin factor B; B-factor, properdin; glycine-rich beta glycoprotein; glycine-rich beta-glycoprotein

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]

Function: 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.

Catalytic activity: Cleavage of Arg-|-Ser bond in complement component C3 alpha-chain to yield C3a and C3b, and Arg-|-Xaa bond in complement component C5 alpha-chain to yield C5a and C5b.

Subunit structure: Monomer.

Subcellular location: Secreted.

Polymorphism: Two major variants, F and S, and 2 minor variants, as well as at least 14 very rare variants, have been identified. The variants His-9 and Gln-32 are associated with a reduced risk of age-related macular degeneration (ARMD) [

MIM:603075]. ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world.

Involvement in disease: Hemolytic uremic syndrome atypical 4 (AHUS4) [MIM:612924]: 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.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. 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. Ref.22 Ref.23

Sequence similarities: Belongs to the peptidase S1 family.Contains 1 peptidase S1 domain.Contains 3 Sushi (CCP/SCR) domains.Contains 1 VWFA domain.

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