Overview

  • Product name

    Anti-Factor H antibody [EPR6225]
    See all Factor H primary antibodies
  • Description

    Rabbit monoclonal [EPR6225] to Factor H
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICCmore details
    Unsuitable for: IHC-P or IP
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide within Human Factor H aa 150-250. The exact sequence is proprietary.

  • Positive control

    • Human plasma, kidney, fetal lung, and fetal liver lysates; Purified Factor H protein.
  • General notes

    Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
  • Dissociation constant (KD)

    KD = 9.40 x 10 -11 M
    Learn more about KD
  • Storage buffer

    pH: 7.40
    Preservative: 0.05% Sodium azide
    Constituents: 40% Glycerol, 9.85% Tris glycine, 50% Tissue culture supernatant
  • Purity

    Tissue culture supernatant
  • Clonality

    Monoclonal
  • Clone number

    EPR6225
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab133536 in the following tested applications.

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

Application Abreviews Notes
WB 1/1000 - 1/10000. Detects a band of approximately 180 kDa (predicted molecular weight: 139 kDa).
ICC 1/50 - 1/100.
  • Application notes
    Is unsuitable for IHC-P or IP.
  • Target

    • Function

      Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway.
    • Tissue specificity

      Expressed by the liver and secreted in plasma.
    • Involvement in disease

      Genetic variations in CFH are associated with basal laminar drusen (BLD) [MIM:126700]; also known as drusen of Bruch membrane or cuticular drusen or grouped early adult-onset drusen. Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early adult-onset drusen phenotype that shows a pattern of uniform small, slightly raised yellow subretinal nodules randomly scattered in the macula. In later stages, these drusen often become more numerous, with clustered groups of drusen scattered throughout the retina. In time these small basal laminar drusen may expand and ultimately lead to a serous pigment epithelial detachment of the macula that may result in vision loss.
      Defects in CFH are the cause of complement factor H deficiency (CFH deficiency) [MIM:609814]. CFH deficiency determines uncontrolled activation of the alternative complement pathway with consumption of C3 and often other terminal complement components. It is associated with a number of renal diseases with variable clinical presentation and progression, including membranoproliferative glomerulonephritis and atypical hemolytic uremic syndrome. CFH deficiency patients may show increased susceptibility to meningococcal infections.
      Defects in CFH are a cause of susceptibility to hemolytic uremic syndrome atypical type 1 (AHUS1) [MIM:235400]. 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.
      Genetic variation in CFH is associated with age-related macular degeneration type 4 (ARMD4) [MIM:610698]. ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.
    • Sequence similarities

      Contains 20 Sushi (CCP/SCR) domains.
    • Cellular localization

      Secreted.
    • Information by UniProt
    • Database links

    • Alternative names

      • adrenomedullin binding protein antibody
      • age related maculopathy susceptibility 1 antibody
      • AHUS 1 antibody
      • AHUS1 antibody
      • AMBP 1 antibody
      • AMBP1 antibody
      • ARMD 4 antibody
      • ARMD4 antibody
      • ARMS 1 antibody
      • ARMS1 antibody
      • beta 1 H globulin antibody
      • beta 1H antibody
      • beta1H antibody
      • CFAH_HUMAN antibody
      • CFH antibody
      • CFHL 3 antibody
      • CFHL3 antibody
      • Complement factor H antibody
      • complement factor H, isoform b antibody
      • Factor H antibody
      • factor H like 1 antibody
      • FH antibody
      • FHL 1 antibody
      • FHL1 antibody
      • H factor 1 (complement) antibody
      • H factor 1 antibody
      • H factor 2 (complement) antibody
      • HF 1 antibody
      • HF 2 antibody
      • HF antibody
      • HF1 antibody
      • HF2 antibody
      • HUS antibody
      • MGC88246 antibody
      see all

    Images

    • All lanes : Anti-Factor H antibody [EPR6225] (ab133536) at 1/1000 dilution

      Lane 1 : Purified Factor H protein at 0.5 µg
      Lane 2 : Purified Factor H protein at 0.1 µg

      Performed under reducing conditions.

      Predicted band size: 139 kDa
      Observed band size: 170 kDa
      why is the actual band size different from the predicted?



      This blot was produced using a 4-12% Bis-tris under the MOPS buffer system. The gel was run at 200V for 55 minutes before being transferred onto a Nitrocellulose membrane at 30V for 70 minutes. The membrane was then blocked for an hour using 3% milk before being incubated with ab133536 overnight at 4°C at a 1/1000 dilution. Antibody binding was detected using Goat anti-Rabbit IgG H&L (IRDye® 800CW) preadsorbed (ab216773) at 1/20000 dilution for 1 hour at room temperature before imaging.

    • All lanes : Anti-Factor H antibody [EPR6225] (ab133536) at 1/1000 dilution

      Lane 1 : Human plasma lysate
      Lane 2 : Human kidney lysate
      Lane 3 : Human fetal lung lysate
      Lane 4 : Human fetal liver lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution

      Predicted band size: 139 kDa
      Observed band size: 180 kDa why is the actual band size different from the predicted?

    • Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD

    References

    This product has been referenced in:

    • Liu W  et al. Molecular basis of coronary artery dilation and aneurysms in patients with Kawasaki disease based on differential protein expression. Mol Med Rep 17:2402-2414 (2018). Read more (PubMed: 29207079) »
    See 1 Publication for this product

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