Invitrogen™ Complement Factor Bb Monoclonal Antibody (10-09)

Mouse Monoclonal Antibody

Supplier:  Invitrogen™ MA528085

Catalog No. PIMA528085

Description

This antibody recognizes the Bb fragment of 90 kDa human complement factor B, present in blood serum. C3b associates with complement factor B, inducing conformational change which 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.

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.

Specifications

• Antigen: Complement Factor Bb
• Classification: Monoclonal
• Concentration: 1.05 mg/mL
• Formulation: BBS with 0.1% sodium azide
• Gene Accession No.: P00751
• Gene Symbols: CFB
• Immunogen: Human factor B.
• Quantity: 100 μg
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG1

• Applications: ELISA, Flow Cytometry, Immunohistochemistry (Frozen), Western Blot
• Clone: 10-09
• Conjugate: Unconjugated
• Gene: CFB
• Gene Alias: AHUS4; ARMD14; BF; B-factor, properdin; BFD; C3 proaccelerator; C3 proactivator; C3/C5 convertase; CFAB; CFB; CFBD; Complement factor B; Complement factor B Ba fragment; Complement factor B Bb fragment; FB; FBI12; GBG; Glycine-rich beta glycoprotein; glycine-rich beta-glycoprotein; H2-Bf; PBF2; properdin factor B
• Host Species: Mouse
• Purification Method: Protein A
• Regulatory Status: RUO
• Gene ID (Entrez): 629
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid