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Mouse Anti-CFHR5 Recombinant Antibody (C1108) (CBMAB-C1108-LY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
C1108
Antibody Isotype
IgG1
Application
WB, FC, ELISA, IHC, IF

Basic Information

Immunogen
Purified recombinant fragment of human Complement Factor H-related 5/CFHR5 (AA: 344-569).
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal Antibody
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
ELISA1:10,000
WB1:500-1:2,000
IF(ICC)1:200-1:1,000
IHC1:200-1:1,000
FC1:200-1:400

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

Format
Liquid
Buffer
PBS
Preservative
0.05% sodium azide
Concentration
1 mg/ml
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 H Related 5
Introduction
This gene is a member of a small complement factor H (CFH) gene cluster on chromosome 1. Each member of this gene family contains multiple short consensus repeats (SCRs) typical of regulators of complement activation. The protein encoded by this gene has nine SCRs with the first two repeats having heparin binding properties, a region within repeats 5-7 having heparin binding and C reactive protein binding properties, and the C-terminal repeats being similar to a complement component 3 b (C3b) binding domain. This protein co-localizes with C3, binds C3b in a dose-dependent manner, and is recruited to tissues damaged by C-reactive protein. Allelic variations in this gene have been associated, but not causally linked, with two different forms of kidney disease: membranoproliferative glomerulonephritis type II (MPGNII) and hemolytic uraemic syndrome (HUS). [provided by RefSeq, Jan 2010]
Entrez Gene ID
81494
UniProt ID
Q9BXR6
Alternative Names
Complement Factor H Related 5; CFHL5; FHR-5; FHR5; Complement Factor H-Related Protein 5; Complement Factor H-Related 5; Factor H Related Protein 5; Factor H-Related Protein 5; CFHR5D;
Function
Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH.
Biological Process
Complement activation, alternative pathway Source: UniProtKB
Cytolysis by host of symbiont cells Source: UniProtKB
Negative regulation of protein binding Source: UniProtKB
Regulation of complement activation Source: Reactome
Cellular Location
Secreted
Involvement in disease
Defects in CFHR5 have been found in patients with atypical hemolytic uremic syndrome and may contribute to the disease. Atypical hemolytic uremic syndrome 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. 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.
CFHR5 deficiency (CFHR5D): A progressive disease characterized by glomerulonephritis, hematuria, renal failure, end-stage renal disease, subendothelial and mesangial glomerular C3 deposits, mesangial matrix expansion, increased glomerular cellularity, and segmental capillary wall thickening. Hematuria may become apparent after respiratory infections.
More Infomation

Gómez Delgado, I., Gutiérrez-Tenorio, J., Fraga Rodríguez, G. M., Cavero, T., Arjona, E., & Sánchez-Corral, P. (2021). Low factor H-related 5 levels contribute to infection-triggered haemolytic uraemic syndrome and membranoproliferative glomerulonephritis. Clinical Kidney Journal, 14(2), 707-709.

Malik, T. H., Gitterman, D. P., Lavin, D. P., Lomax-Browne, H. J., Hiemeyer, E. C., Moran, L. B., ... & Pickering, M. C. (2021). Gain-of-function factor H–related 5 protein impairs glomerular complement regulation resulting in kidney damage. Proceedings of the National Academy of Sciences, 118(13).

Lorés-Motta, L., van Beek, A. E., Willems, E., Zandstra, J., van Mierlo, G., Einhaus, A., ... & den Hollander, A. I. (2021). Common haplotypes at the CFH locus and low-frequency variants in CFHR2 and CFHR5 associate with systemic FHR concentrations and age-related macular degeneration. The American Journal of Human Genetics, 108(8), 1367-1384.

Kadkhodayi-Kholghi, N., Bhatt, J. S., Gor, J., McDermott, L. C., Gale, D. P., & Perkins, S. J. (2020). The solution structure of the complement deregulator FHR5 reveals a compact dimer and provides new insights into CFHR5 nephropathy. Journal of Biological Chemistry, 295(48), 16342-16358.

Hu, X., Liu, H., Du, J., Chen, Y., Yang, M., Xie, Y., ... & Gong, Z. (2019). The clinical significance of plasma CFHR 1–5 in lupus nephropathy. Immunobiology, 224(3), 339-346.

Medjeral-Thomas, N. R., Moffitt, H., Lomax-Browne, H. J., Constantinou, N., Cairns, T., Cook, H. T., & Pickering, M. C. (2019). Glomerular Complement Factor H–Related Protein 5 (FHR5) Is Highly Prevalent in C3 Glomerulopathy and Associated With Renal Impairment. Kidney international reports, 4(10), 1387-1400.

Zhu, L., Guo, W. Y., Shi, S. F., Liu, L. J., Lv, J. C., Medjeral-Thomas, N. R., ... & Zhang, H. (2018). Circulating complement factor H–related protein 5 levels contribute to development and progression of IgA nephropathy. Kidney international, 94(1), 150-158.

Zhang, P., Zhu, M., Geng‐Spyropoulos, M., Shardell, M., Gonzalez‐Freire, M., Gudnason, V., ... & Semba, R. D. (2017). A novel, multiplexed targeted mass spectrometry assay for quantification of complement factor H (CFH) variants and CFH‐related proteins 1–5 in human plasma. Proteomics, 17(6), 1600237.

Togarsimalemath, S. K., Sethi, S. K., Duggal, R., Le Quintrec, M., Jha, P., Daniel, R., ... & Dragon-Durey, M. A. (2017). A novel CFHR1-CFHR5 hybrid leads to a familial dominant C3 glomerulopathy. Kidney international, 92(4), 876-887.

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

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