Complement Factor H Antibody

Product Discontinued

Complement Factor H Antibody Summary

• Immunogen: Human 155kDa Factor H, purified from plasma.
• Localization: Secreted
• Specificity: In IEP a single arc is observed against human plasma. Identity has been confirmed by double diffusion against human plasma and a known anti-human Factor H. Deficiency in factor H can lead to diseases such as Hemolytic-uremic syndrome and chronic hypocomplementemic nephropathy.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Sheep
• Gene: CFH
• Purity: Ion exchange chromatography

Applications/Dilutions

• Dilutions:
  • Immunodiffusion
  • Western Blot 1:100-1:2000
• Application Notes: This antibody is useful for Western Blot (PMID: 21477582)

Reactivity Notes

Reacts with: Guinea Pig, Cat, Mouse, Rat, Dog, Horse.

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: Glycine Buffered Saline (pH 7.4), 0.01% Benzamidine, 0.1% EACA and 1.0mM EDTA
• Preservative: 0.09% Sodium Azide
• Purity: Ion exchange chromatography

Alternate Names for Complement Factor H Antibody

• adrenomedullin binding protein
• age-related maculopathy susceptibility 1
• AHUS1
• AMBP1
• ARMD4
• ARMS1
• beta-1H
• beta-1-H-globulin
• beta-1-H-globulin
• CFH
• CFHL3
• Complement Factor H
• factor H
• factor H-like 1
• FH
• FHL1
• H factor 1 (complement)
• H factor 1
• H factor 2 (complement)
• HF
• HF1
• HF1ARMS1
• HF2
• HUS
• HUSMGC88246

Background

The complement Factor H protein is secreted into the bloodstream and acts in the regulation of complement activation. Mutations leading to changes in this protein have been linked with HUS (hemolytic-uremic syndrome) and chronic hypocomplementemic nephropathy. Factor H is mainly synthesised in the liver but also in macrophages and endothelium. It is primarily aplasma glycoprotein but is also found in platelets and there is a membrane bound form on some leukocytes. Consisting of a single polypeptide, the major form of Factor H has a molecular weight of 155kDa. There are two truncated forms, a non-glycosylated 49 kDa form and a glycosylated 39-43 kDaform. Plasma concentrations are in the range 200-600mg/L for the 155 kDa form and 1-5mg/L for thetruncated forms. Factor H is a major regulatory protein of the complement system. By binding to C3b it either displacesor prevents the binding of Bb (activated Factor B). When bound to Factor H, C3b is susceptible tocleavage by Factor 1 to yield iC3b. Factor H is released or modified following this cleavage. The regulatory role of Factor H is essential because C3bBb is not only a C5 convertase but a C3 convertaseand so has a positive feedback effect, potentially consuming the entire C3 pool if unregulated.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for Complement Factor H Antibody (NB100-62177)(1)

Publication using NB100-62177

• Radu RA, Hu J, Yuan Q et al. Complement system dysregulation and inflammation in the retinal pigment epithelium of a mouse model for stargardt macular degeneration. J Biol Chem;286(21):18593-601. 2011-05-27 [PMID: 21464132]

Bioinformatics

• Gene Symbol: CFH
• Entrez:
  • 3075(Human)
  • 12628(Mouse)
  • 155012(Rat)
• Uniprot:
  • P08603()