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Mouse Anti-CFH Recombinant Antibody (CBXC-0384) (CBMAB-C4151-CQ)

This product is a mouse antibody that recognizes CFH. The antibody CBXC-0384 can be used for immunoassay techniques such as: ELISA, IP, RIA, WB.
See all CFH antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXC-0384
Antibody Isotype
IgG1
Application
ELISA, IP, RIA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.2
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Complement Factor H
Introduction
This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.
Entrez Gene ID
3075
UniProt ID
P08603
Alternative Names
Complement Factor H; Age-Related Maculopathy Susceptibility 1; H Factor 2 (Complement); H Factor 1 (Complement); Beta-1H; HF1; HF2; HF; Adrenomedullin Binding Protein; Beta-1-H-Globulin; Factor H-Like 1;
Function
Glycoprotein that plays an essential role in maintaining a well-balanced immune response by modulating complement activation. Acts as a soluble inhibitor of complement, where its binding to self markers such as glycan structures prevents complement activation and amplification on cell surfaces (PubMed:21285368, PubMed:25402769).
Accelerates the decay of the complement alternative pathway (AP) C3 convertase C3bBb, thus preventing local formation of more C3b, the central player of the complement amplification loop (PubMed:19503104).
As a cofactor of the serine protease factor I, CFH also regulates proteolytic degradation of already-deposited C3b (PubMed:18252712, PubMed:28671664).
In addition, mediates several cellular responses through interaction with specific receptors. For example, interacts with CR3/ITGAM receptor and thereby mediates the adhesion of human neutrophils to different pathogens. In turn, these pathogens are phagocytosed and destroyed (PubMed:9558116, PubMed:20008295).
Biological Process
Complement activation Source: AgBase
Complement activation, alternative pathway Source: UniProtKB-KW
Regulation of complement activation Source: MGI
Regulation of complement-dependent cytotoxicity Source: MGI
Viral process Source: UniProtKB-KW
Cellular Location
Secreted
Involvement in disease
Basal laminar drusen (BLD): 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.
Complement factor H deficiency (CFHD): A disorder that can manifest as several different phenotypes, including asymptomatic, recurrent bacterial infections, and renal failure. Laboratory features usually include decreased serum levels of factor H, complement component C3, and a decrease in other terminal complement components, indicating activation of the alternative complement pathway. It is associated with a number of renal diseases with variable clinical presentation and progression, including membranoproliferative glomerulonephritis and atypical hemolytic uremic syndrome.
Hemolytic uremic syndrome atypical 1 (AHUS1): 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.
Macular degeneration, age-related, 4 (ARMD4): A form of age-related macular degeneration, 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 that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.
PTM
Sulfated on tyrosine residues.

Mao, X., Zhou, L., Tey, S. K., Ma, A. P. Y., Yeung, C. L. S., Ng, T. H., ... & Yam, J. W. P. (2020). Tumour extracellular vesicle‐derived Complement Factor H promotes tumorigenesis and metastasis by inhibiting complement‐dependent cytotoxicity of tumour cells. Journal of extracellular vesicles, 10(1), e12031.

Mühlig, A. K., Keir, L. S., Abt, J. C., Heidelbach, H. S., Horton, R., Welsh, G. I., ... & Oh, J. (2020). Podocytes produce and secrete functional complement C3 and complement factor H. Frontiers in immunology, 11, 1833.

Laskowski, J., Renner, B., Pickering, M. C., Serkova, N. J., Smith-Jones, P. M., Clambey, E. T., ... & Thurman, J. M. (2020). Complement factor H–deficient mice develop spontaneous hepatic tumors. The Journal of clinical investigation, 130(8).

Landowski, M., Kelly, U., Klingeborn, M., Groelle, M., Ding, J. D., Grigsby, D., & Rickman, C. B. (2019). Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation in mice. Proceedings of the National Academy of Sciences, 116(9), 3703-3711.

Shin, C., Ham, B. J., Ko, Y. H., Pae, C. U., Park, M. H., Steffens, D. C., ... & Han, C. (2019). Increased plasma complement factor H is associated with geriatric depression. International psychogeriatrics, 31(1), 101-108.

Toomey, C. B., Johnson, L. V., & Rickman, C. B. (2018). Complement factor H in AMD: Bridging genetic associations and pathobiology. Progress in retinal and eye research, 62, 38-57.

Cantsilieris, S., Nelson, B. J., Huddleston, J., Baker, C., Harshman, L., Penewit, K., ... & Eichler, E. E. (2018). Recurrent structural variation, clustered sites of selection, and disease risk for the complement factor H (CFH) gene family. Proceedings of the National Academy of Sciences, 115(19), E4433-E4442.

Bian, A., Wang, Y., Liu, J., Wang, X., Liu, D., Jiang, J., ... & Hui, X. (2018). Circular RNA complement factor H (CFH) promotes glioma progression by sponging miR-149 and regulating AKT1. Medical science monitor: international medical journal of experimental and clinical research, 24, 5704.

Merinero, H. M., García, S. P., García-Fernández, J., Arjona, E., Tortajada, A., & de Córdoba, S. R. (2018). Complete functional characterization of disease-associated genetic variants in the complement factor H gene. Kidney international, 93(2), 470-481.

Calippe, B., Augustin, S., Beguier, F., Charles-Messance, H., Poupel, L., Conart, J. B., ... & Sennlaub, F. (2017). Complement factor H inhibits CD47-mediated resolution of inflammation. Immunity, 46(2), 261-272.

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For research use only. Not intended for any clinical use.

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