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Product Name Factor B Chinese Name 补体因子B抗体 Alias AHUS4; B factor properdin; Factor B ; BF; BFD; C3 proaccelerator; C3 proactivator; C3/C5 convertase; CFAB; CFAB_HUMAN; CFB; Complement Factor B; Complement factor B Bb fragment; FB; FBI12; GBG; Glycine rich beta glycoprotein; Glycine-rich beta glycoprotein; H2 Bf; H2BF; PBF2; Properdin factor B, literatures Specific References (1) | SL6949R has been referenced in 1 publications.[IF=2.96] Chen C et al. Alternative complement pathway is activated in the brains of scrapie-infected rodents. Med Microbiol Immunol. 2019 Nov 12. IHC-P&IHF ; Mouse&Chinese golden hamsters.Research Area Cell biology immunology Signal transduction Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, Rat, Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 55/81kDa Cellular localization Secretory protein Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Complement factor-B Bb fragment: 301-400/764 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail The complement component proteins, C3, C4 and C5, are potent anaphylatoxins that are released during complement activation. Binding of these proteins to their respective G protein-coupled receptors, C3aR, C1R and C5aR, induces proinflammatory events, such as cellular degranulation, smooth muscle contraction, arachidonic acid metabolism, cytokine release, leukocyte activation and cellular chemotaxis. Complement Factor B, also designated Properdin Factor B or PBF2, is part of the alternate pathway of the complement system and is cleaved by Factor D into two fragments: Ba and Bb. Bb combines with complement Factor 3b to produce the C3 or C5 convertase and plays a role in the differentiation and proliferation of preactivated B lymphocytes, lysis of erythrocytes, stimulation of lymphocyte blastogenesis and rapid spreading of peripheral blood monocytes. Ba is important in inhibiting the proliferation of preactivated B lymphocytes. Adipsin, also designated complement Factor D, is a serine protease that cleaves complement Factor B and may be involved in obesity. Factor H controls the function of the alternative complement pathway. FHR-1 (complement Factor H related protein 1) may play a role in lipid metabolism.
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.
Subunit:
Monomer.
Subcellular Location:
Secreted.
DISEASE:
Defects in CFB are a cause of susceptibility to hemolytic uremic syndrome atypical type 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=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.
Similarity:
Belongs to the peptidase S1 family.
Contains 1 peptidase S1 domain.
Contains 3 Sushi (CCP/SCR) domains.
Contains 1 VWFA domain.
SWISS:
P00751
Gene ID:
629
Database links:Entrez Gene: 629 Human
Entrez Gene: 14962 Mouse
Omim: 138470 Human
SwissProt: P00751 Human
SwissProt: P04186 Mouse
Unigene: 69771 Human
Unigene: 653 Mouse
Unigene: 109148 Rat
Product Picture Sample:
Lane 1: Serum (Rat) at 40 ug
Primary: Anti-Factor B (SL6949R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 93 kD
Observed band size: 100 kD
Paraformaldehyde-fixed, paraffin embedded (mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (GDNF) Polyclonal Antibody, Unconjugated (SL6949R Factor B) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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