Overview

  • Product name

    Anti-Factor H antibody [C3]
    See all Factor H primary antibodies
  • Description

    Mouse monoclonal [C3] to Factor H
  • Host species

    Mouse
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Rat
  • Immunogen

    Recombinant fragment corresponding to Rat Factor H aa 975-1217. N-terminal His tag. Expressed in E. coli.
    Sequence:

    AFIKCVGGQWSEPPKCIKTDCDNLPTFEIAKPTEKKKKSYRSGEQVTFRC PPPYRMDGSDIVTCVNTKWIGQPVCKDNSCVNPPHVPNATILTRHKTKYP SGDKVRYDCNKPFELFGEVEVMCQNGIWTEPPKCKDSTGKCGPPPPIDNG DITSLSLPVYAPLSSVEYQCQNYYLLKGNKIVTCRNGKWSQPPTCLHACV IPEDIMEKHNIVLRWRENAKIYSQSGENIEFMCKPGYRKFRGS


    Database link: G3V9R2

  • Positive control

    • WB: Recombinant rat Factor H protein. Rat cerebrum lysate. Rat serum.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.4
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 50% Glycerol
  • Concentration information loading...
  • Purity

    Protein A/G purified
  • Purification notes

    Purified from TCS.
  • Clonality

    Monoclonal
  • Clone number

    C3
  • Isotype

    IgG2a
  • Light chain type

    kappa
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab239612 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 Use a concentration of 0.5 - 5 µg/ml.

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

  • Anti-Factor H antibody [C3] (ab239612) at 2 µg/ml + Rat serum

    Secondary
    HRP-Goat Anti-Mouse IgG at 0.2 µg/ml
  • Anti-Factor H antibody [C3] (ab239612) at 5 µg/ml + Recombinant rat Factor H protein
  • Anti-Factor H antibody [C3] (ab239612) at 2 µg/ml + Rat cerebrum lysate

    Secondary
    HRP-Goat Anti-Mouse IgG at 0.2 µg/ml

References

ab239612 has not yet been referenced specifically in any publications.

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