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Mouse Anti-CFI Recombinant Antibody (4E9) (CBMAB-C6897-LY)

This product is antibody recognizes CFI. The antibody 4E9 immunoassay techniques such as: WB.
See all CFI antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
4E9
Antibody Isotype
IgG2a
Application
WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2a
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
1% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
1 mg/ml
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Complement Factor I
Introduction
This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by RefSeq, Dec 2015]
Entrez Gene ID
3426
UniProt ID
P05156
Alternative Names
Complement Factor I; Konglutinogen-Activating Factor; C3B/C4B Inactivator; C3b-Inactivator; IF; Complement Control Protein Factor I; Complement Factor I Heavy Chain; Light Chain Of Factor I; Complement Component I; I Factor (Complement);
Function
Trypsin-like serine protease that plays an essential role in regulating the immune response by controlling all complement pathways. Inhibits these pathways by cleaving three peptide bonds in the alpha-chain of C3b and two bonds in the alpha-chain of C4b thereby inactivating these proteins (PubMed:7360115, PubMed:17320177).
Essential cofactors for these reactions include factor H and C4BP in the fluid phase and membrane cofactor protein/CD46 and CR1 on cell surfaces (PubMed:2141838, PubMed:9605165, PubMed:12055245).
The presence of these cofactors on healthy cells allows degradation of deposited C3b by CFI in order to prevent undesired complement activation, while in apoptotic cells or microbes, the absence of such cofactors leads to C3b-mediated complement activation and subsequent opsonization (PubMed:28671664).
Biological Process
Complement activation, classical pathway Source: UniProtKB-KW
Innate immune response Source: UniProtKB-KW
Regulation of complement activation Source: Reactome
Viral process Source: UniProtKB-KW
Cellular Location
Extracellular space; Secreted
Involvement in disease
Hemolytic uremic syndrome atypical 3 (AHUS3): 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.
Complement factor I deficiency (CFI deficiency): Autosomal recessive condition associated with a propensity to pyogenic infections.
Macular degeneration, age-related, 13 (ARMD13): 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.

Jia, B. B., Jin, C. D., & Li, M. F. (2020). The trypsin-like serine protease domain of Paralichthys olivaceus complement factor I regulates complement activation and inhibits bacterial growth. Fish & shellfish immunology, 97, 18-26.

Hallam, T. M., Marchbank, K. J., Harris, C. L., Osmond, C., Shuttleworth, V. G., Griffiths, H., ... & Lotery, A. J. (2020). Rare genetic variants in complement factor I lead to low FI plasma levels resulting in increased risk of age-related macular degeneration. Investigative ophthalmology & visual science, 61(6), 18-18.

Lv, W., Ma, A., Chi, X., Li, Q., Pang, Y., & Su, P. (2020). A novel complement factor I involving in the complement system immune response from Lampetra morii. Fish & shellfish immunology, 98, 988-994.

Altmann, T., Torvell, M., Owens, S., Mitra, D., Sheerin, N. S., Morgan, B. P., ... & Forsyth, R. (2020). Complement factor I deficiency: A potentially treatable cause of fulminant cerebral inflammation. Neurology-Neuroimmunology Neuroinflammation, 7(3).

Yu, Q., Zhu, J., Yao, Y., & Sun, C. (2020). Complement family member CFI polymorphisms and AMD susceptibility from a comprehensive analysis. Bioscience reports, 40(4), BSR20200406.

Shields, A. M., Pagnamenta, A. T., Pollard, A. J., Taylor, J. C., Allroggen, H., Patel, S. Y., ... & Smith, C. (2019). Classical and non-classical presentations of complement factor I deficiency: two contrasting cases diagnosed via genetic and genomic methods. Frontiers in immunology, 10, 1150.

Tseng, M. H., Lin, S. H., Wu, C. Y., Chien, H. P., Yang, H. Y., Chen, Y. C., ... & Huang, J. L. (2018). Serum complement factor I is associated with disease activity of systemic lupus erythematosus. Oncotarget, 9(9), 8502.

Lashkari, K., Teague, G., Chen, H., Lin, Y. Q., Kumar, S., McLaughlin, M. M., & López, F. J. (2018). A monoclonal antibody targeting amyloid β (Aβ) restores complement factor I bioactivity: Potential implications in age-related macular degeneration and Alzheimer’s disease. PLoS One, 13(5), e0195751.

Nanthapisal, S., Eleftheriou, D., Gilmour, K., Leone, V., Ramnath, R., Omoyinmi, E., ... & Brogan, P. A. (2018). Cutaneous vasculitis and recurrent infection caused by deficiency in complement factor I. Frontiers in immunology, 9, 735.

Franco-Jarava, C., de la Campa, E. Á., Solanich, X., Morandeira-Rego, F., Mas-Bosch, V., García-Prat, M., ... & Colobran, R. (2017). Early versus late diagnosis of complement factor I deficiency: clinical consequences illustrated in two families with novel homozygous CFI mutations. Journal of clinical immunology, 37(8), 781-789.

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

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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