CFHR5

Background

CFHR5, also known as complement factor H-related protein 5 or FHR-5, is a 65 kDa protein that is encoded by the CFHR5 gene. It belongs to the factor H-related protein family which consists of the complement and immune regulators factor H, the factor H-like protein 1 (FHL-1), and five factor H-related proteins. Members of this family are exclusively composed of individually folded protein domains called short consensus repeats (SCRs) or complement control modules. The CFHR5 protein consists of 9 SCRs, with the first two repeats having heparin-binding properties, a region within repeats 5-7 possessing texing heparin-binding and C reactive protein (CRP)-binding characteristics, and the C-terminal repeats similar to a complement component 3 b (C3b)-binding domain. This protein acts as a complement activator and plays an important role in the regulation of the alternative complement pathway. It is co-localized with complement C3, binds C3b in a dose-dependent way, and is recruited to tissues damaged by C-reactive protein. CFHR5 also can suppress the C3 convertase and serve as a co-factor for the cleavage of C3b.

The deficiency of CFHR5 is associated with several kidney diseases such as CFHR5 nephropathy. Mutations with loss of function in CFHR5 were detected in patients with the disease CFHR5 nephropathy, which indicates CFHR5 is important in protecting the kidneys from attack by the complement system.

Fig.1 CFHR5 belongs to the human factor H protein family.^{1, 3}

CFHR5 Functional Service

Creative Biolabs provides a comprehensive range of CFHR5-related products, encompassing anti-CFHR5 Antibodies, CFHR5 ELISA Assay Kits and recombinant CFHR5 Proteins, enabling precise detection, quantification, and functional analysis of CFHR5. These high-quality products are all designed to support advanced research in CFHR5-related diseases, immune responses, and protein function studies.

Fig.2 Distribution of CFHR5 levels in pathophysiological clusters of patients.^2,3

CFHR5 functional testing involves assessing the activity and interaction of CFHR5 proteins in biological systems. Methods include ELISA to quantify CFHR5 levels, Western blotting for protein identification, and flow cytometry to analyze CFHR5’s role in immune cell responses. Functional assays, using recombinant CFHR5 proteins and blocking peptides, are essential in studying its impact on the complement system and retinal diseases. Research has shown that CFHR5 dysfunction correlates with conditions like atypical hemolytic uremic syndrome (aHUS), where altered CFHR5 activity leads to impaired complement regulation. By leveraging our products and expertise, scientists can gain valuable insights into the mechanisms underlying CFHR5-related disorders and develop novel therapeutic strategies.

Creative Biolabs provides CFHR5-functional services, including custom assay development, protein analysis, and functional validation, assisting customers with in-depth insights into CFHR5 activity and disease mechanisms. Our services are tailored for precision and reliability.

References

1. Józsi, Mihály. "Factor H family proteins in complement evasion of microorganisms." Frontiers in immunology 8 (2017): 571.
2. Garam, Nóra, et al. "FHR-5 serum levels and CFHR5 genetic variations in patients with immune complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy." Frontiers in Immunology 12 (2021): 720183.
3. Distributed under Open Access license CC BY 4.0. The image was modified by extracting and using only part of the original image.