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Invitrogen
Complement Factor Bb Neoantigen Monoclonal Antibody (032B-22.1.X)
Product Details
MA5-28084
Product Specifications
Species Reactivity
Human
Host/Isotype
Mouse
/ IgG2a
Class
Monoclonal
Type
Antibody
Clone
032B-22.1.X
Immunogen
Purified Human factor B
Conjugate
Form
Liquid
Concentration
1.125 mg/mL
Purification
Protein A
Storage buffer
BBS
Contains
<0.1% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
AB_2745074
Product Specific Information
This antibody recognizes a neoantigen of the Bb fragment of human complement factor B. Factor B is present in blood serum. C3b associates with complement factor B, inducing conformational change. This enables complement factor D to cleave the N-terminal of complement factor B (the Ba subunit) leaving the 63 kDa Bb subunit associated with C3b and forming C3 convertase. Subunit Bb is involved in the proliferation of preactivated B lymphocytes. Conversely, Ba inhibits lymphocyte proliferation.
Target Information
CFB is involved in hemolytic uremic syndrome atypical 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. 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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: B-factor, properdin; C3 proaccelerator; C3 proactivator; C3/C5 convertase; Complement factor B; GBG; Glycine-rich beta glycoprotein; glycine-rich beta-glycoprotein; PBF2; Properdin factor B
Gene Aliases: AHUS4; ARMD14; BF; BFD; CFAB; CFB; CFBD; FB; FBI12; GBG; H2-Bf; PBF2
UniProt ID: (Human) P00751
Entrez Gene ID: (Human) 629
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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