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

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

  • 中文名稱：
    人補體因子H相關蛋白1(CFHR1)酶聯免疫試劑盒
  • 貨號：
    CSB-EL005274HU
  • 規格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 其他：

產品詳情

  • 產品描述：
    人補體因子H相關蛋白1(CFHR1)酶聯免疫試劑盒（CSB-EL005274HU）為競爭法ELISA試劑盒，定量檢測血清、血漿、組織勻漿樣本中的CFHR1含量。CFHR1是一個研究靶點。它可能參與補體系統的調控等生理過程，在維持機體免疫平衡等方面有重要意義。目前研究聚焦于其在疾病發生發展機制中的作用，如與腎臟疾病等相關性，探索其在疾病診斷、治療和發病機制研究的價值。試劑盒檢測范圍為25 ng/mL-1600 ng/mL，該產品適用于科研領域的基礎研究，例如探索CFHR1在補體系統相關疾病中的表達水平變化、評估其在炎癥或免疫調節中的分子機制，或用于生物樣本中CFHR1濃度的定量分析，為疾病機制研究提供實驗支持。試劑盒嚴格匹配標準品濃度梯度，實驗結果穩定可靠，適用于實驗室常規檢測需求。本品僅用于科研，不用于臨床診斷，產品具體參數及操作步驟詳見產品說明書。
  • 別名：
    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
  • 縮寫：
    CFHR1
  • Uniprot No.：
  • 種屬：
    Homo sapiens (Human)
  • 樣本類型：
    serum, plasma, tissue homogenates
  • 檢測范圍：
    25 ng/mL-1600 ng/mL
  • 靈敏度：
    12.5 ng/mL
  • 反應時間：
    1-5h
  • 樣本體積：
    50-100ul
  • 檢測波長：
    450 nm
  • 研究領域：
    Immunology
  • 測定原理：
    quantitative
  • 測定方法：
    Competitive
  • 精密度：
    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.    
                 
  • 線性度：
    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  
  • 回收率：
    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  
                 
                 
  • 標準曲線：
    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    
  • 數據處理：
  • 貨期：
    3-5 working days

產品評價

靶點詳情

  • 功能：
    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.
  • 基因功能參考文獻：
    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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  • 亞細胞定位：
    Secreted.
  • 組織特異性：
    Expressed by the liver and secreted in plasma.
  • 數據庫鏈接：

    HGNC: 4888

    OMIM: 134371

    KEGG: hsa:3078

    STRING: 9606.ENSP00000314299

    UniGene: Hs.575869



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