Mouse anti-Human Complement factor B/Bb Monoclonal Antibody | anti-CFB antibody

Complement factor B/Bb, Human, mAb M13/12

Cat. Num. AAA584185

• Gene Names: CFB; BF; FB; BFD; GBG; CFAB; CFBD; PBF2; AHUS4; FBI12; H2-Bf; ARMD14
• Reactivity: Human
• Applications: Immunoprecipitation, Western Blot
• Synonyms: Complement factor B/Bb; Monoclonal Antibody; Human; mAb M13/12; anti-CFB antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: Mouse IgG1
• Form/Format: 1 ml (100 ug/ml) 0.2 um filtered antibody solution in PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide.; For unit size 0.5 mg or larger: PBS only (concentration< 0.5mg/ml)
• Sequence Length: 764

• Applicable Applications for anti-CFB antibody: Immunoassay (IA), Immunoprecipitation (IP), Western Blot (WB)
• Application Notes: Application Use: For Western blotting dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.
• Preparation and Storage: Product should be stored at 4 degree C. Under recommended storage conditions, product is stable for one year.

Related Product Information for anti-CFB antibody

Product Description: The monoclonal antibody M13/12 recognizes human complement factor B/Bb. Factor B is an acute-phase protein. Levels of factor B increase during inflammation. Complement factor B is a single- chain molecule of 764 amino acids (MW of 90 kD), including a leader peptide of 25 amino acids. Complement factor B provides the catalytic subunit of the C3/C5 convertases of the alternative complement pathway. Assembly of the C3 convertase (C3bBb) requires binding of complement factor B to C3b (C3.H2O) and factor D-mediated cleavage of bound complement factor B resulting in the release of Ba (MW 33 kD). The C3 convertase is stabilized by the binding of properdin. This provides a positive amplification loop for the classical and alternative complement pathways. Bb (MW 60 kD) is the serine protease element of this convertase. After cleavage of C3, the C5 convertase ((C3b)2Bb) is formed. The Bb fragment may be regarded as a better indicator of the alternative pathway of complement activation than Ba as impaired renal filtration does directly influence Ba levels. Whereas Bb is elevated 2.2-fold in chronic renal failure (CRF) patients, plasma concentrations of Ba are 8.4-fold higher in CRF and 16-fold higher in end-stage renal disease (ESRD) patients compared to normals. In addition to complement activation, factor B fragments participate in other immunological functions.

References

1. Oppermann, M et al; Complement in patients with renal failure as detected through the quantitation of fragments of the complement proteins C3, C5 and factor B. Klin Wochenschr 1988, 66: 857 2. Oppermann, M et al; Quantitaion of components of the alternative pathway of complement (APC) by enzyme-linked immunosorbent assays. J Immunol Methods 1990, 133: 181
3. Oppermann, M et al; Elevated plasma levels of the immunosuppressive complement fragment Ba in renal failure. Kidney Int 1991, 40: 939

NCBI and Uniprot Product Information

• NCBI GI #: 67782358
• NCBI GeneID: 629
• NCBI Accession #: NP_001701.2
• NCBI GenBank Nucleotide #: NM_001710.5
• Molecular Weight: 68,872 Da
• NCBI Official Full Name: complement factor B preproprotein
• NCBI Official Synonym Full Names: complement factor B
• NCBI Official Symbol: CFB
• NCBI Official Synonym Symbols: BF; FB; BFD; GBG; CFAB; CFBD; PBF2; AHUS4; FBI12; H2-Bf; ARMD14
• NCBI Protein Information: complement factor B; C3 proactivator; C3/C5 convertase; C3 proaccelerator; properdin factor B; B-factor, properdin; glycine-rich beta glycoprotein; glycine-rich beta-glycoprotein
• UniProt Protein Name: Complement factor B
• Protein Family: Complement factor
• UniProt Gene Name: CFB
• UniProt Synonym Gene Names: BF; BFD; GBG
• UniProt Entry Name: CFAB_HUMAN

NCBI Description

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]

Uniprot Description

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; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 6p21.3

Cellular Component: extracellular space; extracellular region; plasma membrane

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