Complement Factor H Antibody (028B-244.2.10X (10-10)) [PE]

• Reactivity: Hu
• Applications: WB, ELISA, Flow, IHC
• Clone: 028B-244.2.10X (10-10)
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: PE

Complement Factor H Antibody (028B-244.2.10X (10-10)) [PE] Summary

• Description: This conjugate is made on demand and is not held in inventory. Each order represents a unique lot. All conjugates are generated using validated conjugation protocols and meet strict degree of labeling (F/P) specifications; additional information is available upon request. The antibody concentration is provided on the individual product label.
  
  For specialized conjugation needs, like large quantities, specific concentrations, and defined F/P ratios, bulk and custom antibody conjugation services are available.
• Immunogen: Purified human complement factor H from serum
• Specificity: NB100-64342 recognizes human complement factor H which exists in 2 forms. The most common form, of 150 kDa, and the less common form of 43 kDa. Factor H is secreted by the liver into the blood serum. It is important in regulating the complement pathway, preventing unnecessary inflammation which can damage the host tissue. Complement factor H functions as a cofactor in the inactivation of C3b by factor I. It makes C3b susceptible to cleavage by factor I, resulting in iC3b. Factor H also inhibits the formation of the C3bBb complex (C3 convertase) and increases the rate of dissociation of both C3 convertase and the (C3b)NBB complex (C5 convertase). This prevents these components of the classical and of alternative complement pathways from forming a positive feedback loop. Mutations in factor H are associated with hemolytic-uremic syndrome, age-related macular degeneration, membranoproliferative glomerulonephritis (MPGN) type II and chronic hypocomplementemic nephropathy.
• Isotype: IgG1
• Clonality: Monoclonal
• Host: Mouse
• Gene: CFH
• Purity: Protein A purified

Applications/Dilutions

• Dilutions:
  • ELISA
  • Flow Cytometry
  • Immunohistochemistry
  • Immunohistochemistry-Frozen
  • Western Blot
• Application Notes: Recommended applications are based on validated applications from the unconjugated base product NBP2-52743. This conjugated antibody is not kept in inventory and is made to order using validated conjugation protocols.

Reactivity Notes

Reacts with Human.

Packaging, Storage & Formulations

• Storage: Store at 4C in the dark.
• Buffer: PBS
• Preservative: 0.05% Sodium Azide
• Purity: Protein A purified

Alternate Names for Complement Factor H Antibody (028B-244.2.10X (10-10)) [PE]

• 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.

Bioinformatics

• Gene Symbol: CFH