Anti-Factor B antibody [6G11] (ab17927)
Key features and details
- Mouse monoclonal [6G11] to Factor B
- Reacts with: Human
- Isotype: IgG1
Overview
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Product name
Anti-Factor B antibody [6G11]
See all Factor B primary antibodies -
Description
Mouse monoclonal [6G11] to Factor B -
Host species
Mouse -
Species reactivity
Reacts with: Human -
Immunogen
Factor B isolated from human plasma.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. -
Storage buffer
pH: 7.40
Preservative: 0.097% Sodium azide
Constituents: 0.0268% PBS, 2.9% Sodium chloride -
Concentration information loading...
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Purity
Protein G purified -
Clonality
Monoclonal -
Clone number
6G11 -
Myeloma
x63-Ag8.653 -
Isotype
IgG1 -
Light chain type
kappa -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Target
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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. -
Involvement in 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. -
Sequence similarities
Belongs to the peptidase S1 family.
Contains 1 peptidase S1 domain.
Contains 3 Sushi (CCP/SCR) domains.
Contains 1 VWFA domain. -
Cellular localization
Secreted. - Information by UniProt
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Database links
- Entrez Gene: 629 Human
- Omim: 138470 Human
- SwissProt: P00751 Human
- Unigene: 69771 Human
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Alternative names
- AHUS4 antibody
- B factor properdin antibody
- BF antibody
see all
References (0)
ab17927 has not yet been referenced specifically in any publications.