Human Complement factor H-related protein 1(CFHR1) ELISA kit

Instructions
Code CSB-EL005274HU
Size 96T,5×96T,10×96T
Trial Size 24T ELISA kits trial application
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Product Details

Target Name complement factor H-related 1
Alternative Names CFHL ELISA Kit; CFHL1 ELISA Kit; CFHL1P ELISA Kit; CFHR1 ELISA Kit; CFHR1P ELISA Kit; Complement factor H related 1 ELISA Kit; Complement factor H related 1 pseudogene ELISA Kit; Complement factor H related protein 1 precursor ELISA Kit; Complement factor H-related protein 1 ELISA Kit; FHR 1 ELISA Kit; FHR-1 ELISA Kit; FHR1 ELISA Kit; FHR1_HUMAN ELISA Kit; H factor (complement) like 1 ELISA Kit; H factor (complement) like 2 ELISA Kit; H factor like protein 1 ELISA Kit; H factor-like protein 1 ELISA Kit; H-factor-like 1 ELISA Kit; H36 1 ELISA Kit; H36 2 ELISA Kit; H36 ELISA Kit; HFL1 ELISA Kit; HFL2 ELISA Kit; MGC104329 ELISA Kit
Abbreviation CFHR1
Uniprot No. Q03591
Species Homo sapiens (Human)
Sample Types serum, plasma, tissue homogenates
Detection Range 25 ng/mL-1600 ng/mL
Sensitivity 12.5 ng/mL
Assay Time 1-5h
Sample Volume 50-100ul
Detection Wavelength 450 nm
Research Area Immunology
Assay Principle quantitative
Measurement Competitive
Precision
Intra-assay Precision (Precision within an assay): CV%<8%      
Three samples of known concentration were tested twenty times on one plate to assess.  
Inter-assay Precision (Precision between assays): CV%<10%      
Three samples of known concentration were tested in twenty assays to assess.    
             
Linearity
To assess the linearity of the assay, samples were spiked with high concentrations of human CFHR1 in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
  Sample Serum(n=4)  
1:100 Average % 98  
Range % 92-106  
1:200 Average % 95  
Range % 91-99  
1:400 Average % 102  
Range % 98-106  
1:800 Average % 85  
Range % 80-89  
Recovery
The recovery of human CFHR1 spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
Sample Type Average % Recovery Range  
Serum (n=5) 92 88-98  
EDTA plasma (n=4) 102 98-108  
             
             
Typical Data
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
ng/ml OD1 OD2 Average    
1600 0.089 0.090 0.090    
800 0.164 0.167 0.166    
400 0.279 0.288 0.284    
200 0.491 0.502 0.497    
100 0.754 0.740 0.747    
50 1.068 1.059 1.064    
25 1.545 1.447 1.496    
0 2.521 2.623 2.572    
Troubleshooting
and FAQs
ELISA kit FAQs
Storage Store at 2-8°C. Please refer to protocol.
Lead Time 7-14 working days

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Target Data

Function Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH. Can associate with lipoproteins and may play a role in lipid metabolism.
Gene References into Functions
  1. Novel genetic rearrangement generated from a heterozygous deletion spanning 146 Kbp involving multiple CFHR genes leading to a CFHR1-R5 hybrid protein. This deletion was found in four family members presenting with a familial dominant glomerulopathy. PMID: 28729035
  2. Plasma FHR-1 and the FHR-1/fH ratio were elevated in IgA nephropathy and associated with progressive disease. PMID: 28673452
  3. A role of FHR-1 in IgA nephropathy pathogenesis is to compete with complement regulation by factor H. PMID: 28637589
  4. We conclude that the relationship between complement-regulatory proteins CFHR1 and CFHR3 and response to anti-CD20 mAb therapy varies based on the specific anti-CD20 mAb used. PMID: 27528699
  5. 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
  6. These results identify C-reactive protein as a ligand for FHR-1 and suggest that FHR-1 enhances, rather than inhibits, complement activation, which may explain the protective effect of FHR-1 deficiency in age-related macular degeneration. PMID: 28533443
  7. The CFHR1 level in plasma of T2DM patients were significantly higher than that of the healthy controls. PMID: 28604974
  8. CFHL-1 is a CFH gene splice variant. PMID: 27814381
  9. We investigated whether the causal sequence variant resides in the CFH gene or the neighboring complement factor H-related 1 (CFHR1) gene, and fine mapping Implicates a deletion of CFHR1 in protection from IgA nephropathy in Han Chinese PMID: 26940089
  10. Next-generation sequencing of the CFH region identified putatively functional variants (missense, splice site and indel) on the four common haplotypes. We found no expression of any of the five CFH-related genes in the retina or RPE/Choroid/Sclera, in contrast to the liver, which is the main source of the circulating proteins. [CFHR1] PMID: 27196323
  11. Studies indicate that complement factor H-related proteins (FHR1-5) may enhance complement activation, with important implications for the role of these proteins in disease. PMID: 25979655
  12. These results suggest that the combination of quantitative and qualitative variations in the complement proteins encoded by CFH, CFHR3 and CFHR1 genes is key for the association of these haplotypes with disease. PMID: 26163426
  13. Genetic variants in CFH, CFHR3, and CFHR1 affect complement activation and thereby predispose patients to develop IgA nephropathy. PMID: 25205734
  14. An average of 15.2% of factor H-autoantibody positive individuals with rheumatic diseases or hemolytic uremic syndrome had homozygous deficiency of CFHR1. PMID: 22894814
  15. Data indicate that the autoantigenic epitope of complement factor H (CFH) and its homologous site in CFH-related protein-1(CFHR1) are structurally different PMID: 25659429
  16. Prompt use of immunosuppressive agents and plasma exchanges are useful for improving outcomes in pediatric patients with anti-complement factor H-associated HUS. PMID: 24088957
  17. Atypical haemolytic-uraemic syndrome due to heterozygous mutations of CFH/CFHR1-3 and complement factor H 479. PMID: 24333077
  18. Factor H and the alternative spliced product FHL-1 are expressed in RPE cells. PMID: 24280833
  19. we have assessed the relationship between GA and previously identified AMD-associated variants of genes (CFH, CFB, C3, FHR1, FRH3, and ARMS2/HTRA). PMID: 24557084
  20. Results show non-coding CFH SNP (rs6677604) and the common deletion CNP147 were strongly correlated both with each other and with plasma CFH and CFHR1 concentrations in patients with age-related macular degeneration. PMID: 23873044
  21. The CFHR1/complement factor H hybrid gene fusion protein contains the first three short consensus repeats of CFHR1 PMID: 23880784
  22. These results provide evidence for a role of CFH and FHL-1 in cutaneous squamous cell carcinoma progression and identify them as progression markers and potential therapeutic targets. PMID: 23938460
  23. Homozygous deletion in CFHR1 is strongly associated with occurrence of CFH antibodies in pediatric patients with atypical hemolytic uremic syndrome. PMID: 23243267
  24. Identification and characterization of a unique CFHR1 mutation provides insights into the biology and pathogenic mechanisms underlying C3 glomerulopathy. PMID: 23728178
  25. Lpd is a novel surface-exposed virulence factor of P. aeruginosa that binds Factor H, FHL-1, CFHR1, and plasminogen, and the Lpd-attached regulators are relevant for innate immune escape and most likely contribute to tissue invasion. PMID: 23071278
  26. genetic variations in CFH and its related genes may contribute to hypertension risk in Chinese Hans PMID: 22848687
  27. A hybrid CFHR3-1 gene causes familial C3 glomerulopathy. PMID: 22626820
  28. we show that native factor H, factor H-like protein 1, and factor H-related protein 1 (CFHR1) bind to PTX3 PMID: 22786770
  29. A significant association with deletion of CFHR1-4 was identified in patients who presented with bilateral geographic atrophy. PMID: 22558131
  30. Reduced expression of the CFHR1 allele has been associated with higher risk to atypical Haemolytic Uraemic Syndrome in Spanish patients. PMID: 22136554
  31. Analysis of the CFHR1 genotypes provide sufficient information to delineate the individual risk of developing age-related macular degeneration. PMID: 22247456
  32. Data show that 698 CNPs loci overlap with known disease-associated or pharmacogenetic-related genes such as CFHR3, CFHR1, GSTTI and UGT2B17. PMID: 21677662
  33. In this matched subset of Age-Related Eye Disease Study (AREDS) subjects, after adjusting for 2 known risk variants in CFH, CNP147 deletion statistically associates with diminished risk for AMD. PMID: 21856016
  34. Anti-factor H autoantibodies in patients with autoimmune form of atypical hemolytic uremic syndrome crossreact with CFHR1. PMID: 21677636
  35. Combined deletion of CFHR3 and CFHR1 is associated with a decreased risk of developing age-related macular degeneration. PMID: 21850184
  36. A change in gene dosage of the encoded proteins CFHR3 and CFHR1 might account for the increased systemic lupus erythematosus. risk PMID: 21637784
  37. deficiency of CFHR3 and CFHR1 results in a loss of complement control but enhances local regulation by factor H; alludes to critical balance between CFHR3, CFHR1 and factor H and emphasize role of complement regulation in age-related macular degeneration PMID: 20843825
  38. Studies indicated that atypical HUS was linked with a complement alternative pathway dysregulation due to genetic defects but also to development of autoantibodies to factor H (FH). PMID: 20865640
  39. Binding of the human complement regulators CFHR1 and factor H by streptococcal collagen-like protein 1 (Scl1) via their conserved C termini allows control of the complement cascade at multiple levels. PMID: 20855886
  40. Data from haplotype analysis demonstrates the relationship between the CFH rs10737680 association and the CFHR1-3Delta association in age-related macular degeneration. PMID: 20581873
  41. CFH and CFHR1, when bound on the surface of C. albicans, enhance antimicrobial activity of human neutrophils. PMID: 20008295
  42. Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome. PMID: 19861685
  43. Human complement regulatory factor H-like protein 1 (FHL-1) binds to Streptococcal pyogenes M18 surface protein Emm18. PMID: 15557185
  44. the primary function of FHL-1 binding by T. denticola might be to facilitate adherence to FHL-1 present on anchorage-dependent cells and in the extracellular matrix PMID: 16239506
  45. Deletion of two closely related genes, complement factor H-related 1 (CFHR1) and complement factor H-related 3 (CFHR3), increases the risk of atypical hemolytic uremic syndrome. PMID: 17367211
  46. we have identified factor H-related protein 1 (FHR-1) as a novel protein that binds to Borrelia burgdorferi via CRASP-3, -4, and -5. PMID: 17538892
  47. either lacked the CFHR1/CFHR3 completely (n = 14) or showed extremely low CFHR1/CFHR3 plasma levels (n = 2) are positive for factor H (CFH) autoantibodies PMID: 18006700
  48. The binding of factor H and factor H-like protein 1 (FHL-1) from human sera to Aspergillus fumigatus conidia was shown by adsorption assays and immunostaining. PMID: 18039838
  49. Deletion of CFHR1 and CFHR3 may account for a small portion of the protection from age-related macular degeneration associated with particular haplotypes in complement factor H. PMID: 18084039
  50. CFH/CFHL1 binding site within borrelial BbCRASP-2 and identified single amino acid residues potentially involved in the interaction with both complement regulators PMID: 18824548

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Subcellular Location Secreted
Tissue Specificity Expressed by the liver and secreted in plasma.
Database Links

HGNC: 4888

OMIM: 134371

KEGG: hsa:3078

STRING: 9606.ENSP00000314299

UniGene: Hs.575869

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