Human complement factor H,CFH ELISA Kit

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. 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. As a cofactor of the serine protease factor I, CFH also regulates proteolytic degradation of already-deposited C3b. 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.

1. factors other than the CFH Y402H polymorphism may be involved in the progression of neovascular age-related macular degeneration after treatment with anti-VEGF agents, in Malaysian population PMID: 29579408
2. Functional characterization of disease-associated genetic variants in the complement factor H gene in atypical hemolytic uremic syndrome and C3-glomerulopathy patients. PMID: 28941939
3. Evasion of C3b deposition at division septa and lateral amplification underneath the capsule requires localization of the FH-binding protein PspC at division sites. PMID: 30139996
4. These findings reveal that the CRP- and PTX3-binding characteristics of FHL-1 differ from those of FH, likely underpinning independent immune regulatory functions in the context of the human retina. PMID: 29374201
5. Two rare age-related macular degeneration-associated variants in the CFH gene (rs121913059 [p.Arg1210Cys] and rs35292876) deviate in frequency among different geographic regions. PMID: 29410599
6. these data demonstrate that Mesenchymal Stem Cells inhibit the activation of pathogenic C5 via up-regulation of FH, which improves our understanding of the immunomodulatory mechanisms of MSCs in the treatment of lupus nephritis. PMID: 29885865
7. Mutation in CFH gene is associated with age-related macular degeneration. PMID: 29686068
8. antioxidant and zinc nutritional supplement modifies risk of macular degeneration progression according to genotype PMID: 29311295
9. CC rs1061170 CFH genotype may be associated with the age-related macular degeneration. Additionally, CC rs1061170 CFH genotype may promote a negative response to anti-VEGF treatment, while patients with TT rs1061170 CFH genotype showed better functional and structural response to anti-VEGF agents. PMID: 29912491
10. Our analysis showed stronger contribution of ARMS2 in age-related macular degeneration (AMD) with reticular pseudodrusen (RPD) group versus AMD without RPD group, in comparison with CFH genotypes. PMID: 28593728
11. in Chinese lupus nephritis patients, the variants in the FH gene might affect the histopathologic subtypes and some clinical features of the disease PMID: 28403670
12. Study demonstrated that a novel complotype composed of CFB (rs4151667) in combination with CFB (rs641153) and CFH(rs800292) is strongly associated with complement activation and age-related macular degeneration status. PMID: 27241480
13. The rs193053835 single nucleotide polymorphism showed the most significant protective effect to be associated with susceptibility to Meningococcal disease. PMID: 27805046
14. Mapping rare, deleterious mutations in Factor H: Association with early onset, drusen burden, and lower antigenic levels in familial AMD. PMID: 27572114
15. AMD patients had significantly elevated nitrated CFH levels compared to controls (p = 0.0117). These findings strongly suggest that nitrated CFH contributes to AMD progression, and is a target for therapeutic intervention. PMID: 28159936
16. inhibition of the alternative pathway by factor H, with a concentration equivalent to a high physiological level, strongly reduced C5a levels and decreased proinflammatory cytokine production in human peripheral blood mononuclear cells. PMID: 27721145
17. Complement factor H Y402H (rs1061170) and age-related maculopathy susceptibility2 (ARMS2)/LOC387715 A69S (rs10490924) polymorphisms shown to have significant association with age-related macular degeneration (Meta-Analysis). PMID: 27269047
18. Regression analysis showed that ARMS2 TT genotype has a statistically significant effect on retinal angiomatous proliferation versus age-related macular degeneration compared to CFH genotypes (P < 0.001). PMID: 28005184
19. This study enclosed strong synergistic association of risk genotypes of C3 and CFH Y402H with AMD. We also revealed synergistic influence of CCL2-2518 and the at-risk genotype of the C3 in AMD with an estimated AP = 50.9% (adjusted AP = 24.7%). Present findings show that CCL2-2518 polymorphism is not an innocent bystander in AMD susceptibility when combined with the at-risk genotype of C3 (R102G). PMID: 28095095
20. Our results suggest that factor H can interfere with mycobacterial entry into macrophages and modulate inflammatory cytokine responses, particularly during the initial stages of infection, thus affecting the extracellular survival of the pathogen. PMID: 27262511
21. To our knowledge, this is the first evaluation of the involvement of the CFHR3/CFHR1 deletion and age-related macular degeneration in CFH Y402H polymorphism Brazilian patients. PMID: 26942649
22. The findings of the present study provide evidence that CFH gene variants and ARMS2/HTRA1 genes play a major role in the genetic susceptibility to AMD in a Greek population. These findings are of direct relevance for disease and help mapping the genetic chart of AMD. PMID: 26848857
23. Our results suggest the contribution of all four predicted CFH polymorphisms in age-related macular degeneration (AMD) susceptibility among the Iranian population. This association with CFH may lead to early detection and new strategies for prevention and treatment of AMD. PMID: 25612476
24. Development of polypoidal choroidal vasculopathy (PCV) in the unaffected fellow eye is associated with ARMS2 A69S genotype in patients with unilateral PCV. PMID: 26332911
25. Identification of rare CFH variant carriers may be important for upcoming complement-inhibiting therapies. Patients with an extensive drusen area, drusen with crystalline appearance, and drusen nasal to the optic disc are more likely to have a rare variant in the CFH gene. PMID: 28859202
26. C-reactive protein amino acids 35-47 mediate the interaction with complement factor H in lupus nephritis PMID: 28566480
27. OCT scans revealed lower retinal thickness in patients homozygous for CFH or ARMS2, which was caused by a significantly reduced photoreceptor layer. The number and ultrastructure of drusen were also significantly different. PMID: 28558370
28. VEGF inhibition decreases local CFH and other complement regulators in the eye and kidney through reduced VEGFR2/PKC-alpha/CREB signaling. PMID: 27918307
29. CFH rs1061170 has an important effect on age at onset of MDD in Han Chinese and may therefore be related to early pathogenesis of MDD. PMID: 26941266
30. Data suggest that disease-linked mutations in complement factor H (CFH) affect pivotal role of CFH in regulation of complement activation; mutations studied include those linked to atypical hemolytic uremic syndrome and age-related macular degeneration. PMID: 28637873
31. Factor I binds C3b-Factor H between Factor H domains 2 and 3 and a reoriented C3b C-terminal domain and docks onto the first scissile bond, while stabilizing its catalytic domain for proteolytic activity. PMID: 28671664
32. The VEGF haplotype TGA could be used as a marker for poor visual prognosis in Tunisian patients with neovascular AMD treated with bevacizumab. PMID: 27116510
33. Two protective, low-frequency, non-synonymous variants were significantly associated with a decrease in age-related macular degeneration (AMD)risk: A307V in PELI3 and N1050Y in CFH .We also identified a strong protective signal for a common variant (rs8056814) near CTRB1 associated with a decrease in AMD risk (logistic regression: OR = 0.71, P = 1.8 x 10-07). PMID: 28011711
34. this study shows that RNA interference of factor H in dendritic cells increased alloantigen-specific T-cell proliferation PMID: 28105653
35. monomeric CRP (mCRP) but not the pentameric form (pCRP) upregulates IL-8 and CCL2 levels in retinal pigment epithelial cells. Complement factor H (FH) binds mCRP to dampen its proinflammatory activity. FH from AMD patients carrying the "risk" His402 polymorphism displays impaired binding to mCRP. PMID: 26961257
36. The uromodulin-CFH interaction enhanced the cofactor activity of CFH for factor I-mediated cleavage of C3b to inactivated C3b. PMID: 27113631
37. Our results revealed that SNPs CD59-rs831626 and CFH-rs1065489 were associated with the susceptibility of acute anterior uveitis. PMID: 27419833
38. Our results show that age-related macular degeneration donors carrying the high risk allele for CFH (C) had significantly more mtDNA damage compared with donors having the wild-type genetic profile. PMID: 26854823
39. Data suggest that Staphylococcus aureus surface protein SdrE (serine-aspartate repeat protein E) functions as a 'clamp' to capture C-terminal tail of human CFH (complement factor H) at a specific ligand-binding site/groove via unique close-dock-lock-latch mechanism and thereby sequesters CFH on surface of S. aureus for complement evasion (immune evasion). PMID: 28258151
40. The haplotypic coinheritance of potentially functional variants (including missense variants, novel splice sites, and the CFHR3-CFHR1 deletion) was described for the four common haplotypes. Expression of the short and long CFH transcripts differed markedly between the retina and liver. PMID: 27196323
41. Data suggest that complement factor H (CFH) attaches to surface of host cells to inhibit complement activation and amplification, preventing destruction of the host cells; SdrE (serine-aspartate repeat protein) of Staphylococcus aureus binds to CFH allowing S. aureus to mimic a host cell and reducing bacterial killing by granulocytes. [Commentary] PMID: 28490660
42. Interaction effects between supplement groups and individual complement factor H (CFH) Y402H and age-related maculopathy susceptibility 2 (ARMS2) genotypes, and composite genetic risk groups combining the number of risk alleles for both loci, were evaluated for their association with progression PMID: 27471039
43. EMD were not AMD-independently associated with CFH or ARMS2 genotypes. Our results indicate that patients without AMD but with EMD can serve as controls in studies evaluating AMD risk factors. PMID: 26614632
44. Functional activities of FH are deficient in patients with ANCA-positive vasculitis. PMID: 27939215
45. role for serum FH levels in the host response to invasive pneumococcal infections PMID: 26802141
46. Despite limited power of this pilot study, our results suggest an association of HF with polymorphisms in ARMS2/HTRA1, CFH, APOE4/TOMM40, and VEGFA genes which could be triggered by modification of the extracellular matrix, altered complement system or lipid metabolism. PMID: 27552409
47. data suggest that R1210C is a unique C-terminal complement factor H mutation that behaves as a partial complement factor H deficiency, predisposing individuals to diverse pathologies with distinct underlying pathogenic mechanisms; the final disease outcome is then determined by R1210C-independent genetic risk factors PMID: 26376859
48. AMD progression rate is influenced by CFH, and suggest that variants within CFH may have different effects on risk versus progression. However, since CFH:rs10737680 was not significant after Bonferroni correction and explained only a relatively small portion of variation in progression rate beyond that explained by age PMID: 27832277
49. We describe a novel CFH/CFHR3 hybrid gene secondary to a de novo 6.3-kb deletion that arose through microhomology-mediated end joining rather than nonallelic homologous recombination. We confirmed a transcript from this hybrid gene and showed a secreted protein product that lacks the recognition domain of factor H and exhibits impaired cell surface complement regulation PMID: 26490391
50. The footprint of C3b on the FH surface matches existing crystal structures of C3b complexed with the N- and C-terminal fragments of FH. In addition, data revealed the position of the central portion of FH in the protein complex. Moreover, cross-linking studies confirmed the involvement of the C-terminus in the dimerization of FH. PMID: 27099340

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Basal laminar drusen (BLD); Complement factor H deficiency (CFHD); Hemolytic uremic syndrome atypical 1 (AHUS1); Macular degeneration, age-related, 4 (ARMD4)

Secreted.

Expressed in the retinal pigment epithelium (at protein level). CFH is one of the most abundant complement components in blood where the liver is the major source of CFH protein in vivo. in addition, CFH is secreted by additional cell types including mono

HGNC: 4883

OMIM: 126700

KEGG: hsa:3075

STRING: 9606.ENSP00000356399

UniGene: Hs.363396