Recombinant Human Factor B protein (ab73657)

Overview

Description

  • Nature
    Recombinant
  • Source
    EBNA 293 cells
  • Amino Acid Sequence
    • Species
      Human

Specifications

Our Abpromise guarantee covers the use of ab73657 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Functional Studies

  • Purity
    > 95 % SDS-PAGE.
    1. IMAC using nickel sepharose FF (25mM tris 500mM NaCl pH 8.2, 10-500 mM imidazol step-gradient). 2. Anionic exchange using ResourceQ (25mM tris pH 8.0, 25-1000mM NaCl linear-gradient). 3. Gel filtration using Superdex200 (25mM Tris 150mM NaCl ph7.5).
  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Preservative: None
    Constituents: 25mM Tris, 150mM Sodium chloride, pH 7.5

General Info

  • Alternative names
    • AHUS4
    • B factor properdin
    • 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
    see all
  • 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

Images

  • SDS-PAGE of purified recombinant factor B treated and untreated with PNGaseF.
  • NuPage analysis of purified factor B at 4 dilutions under non-reducing and reducing conditions.

References

ab73657 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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