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  4. Human Complement factor H-related protein 3(CFHR3) ELISA kit

Human Complement factor H-related protein 3(CFHR3) ELISA kit

  • 中文名稱：
    人補(bǔ)體因子H相關(guān)蛋白3(CFHR3)酶聯(lián)免疫試劑盒
  • 貨號：
    CSB-EL005276HU
  • 規(guī)格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品描述：
    人補(bǔ)體因子H相關(guān)蛋白3(CFHR3)酶聯(lián)免疫試劑盒（CSB-EL005276HU）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿樣本中的CFHR3含量。人補(bǔ)體因子H相關(guān)蛋白3（CFHR3）是一種補(bǔ)體調(diào)節(jié)蛋白，與補(bǔ)體系統(tǒng)功能密切相關(guān)。其基因變異與腎臟疾病如非典型溶血性尿毒癥綜合征和C3腎小球病有關(guān)，通過調(diào)節(jié)補(bǔ)體功能影響疾病發(fā)生發(fā)展。試劑盒檢測范圍為31.25 ng/mL-2000 ng/mL，本產(chǎn)品可廣泛應(yīng)用于體外實(shí)驗(yàn)研究，包括補(bǔ)體系統(tǒng)功能解析、疾病模型建立中的生物標(biāo)志物檢測以及免疫調(diào)節(jié)相關(guān)分子機(jī)制探索等科研場景，為探究CFHR3在炎癥反應(yīng)、免疫復(fù)合物清除等過程中的動態(tài)變化提供可靠工具。本品僅用于科研，不用于臨床診斷，產(chǎn)品具體參數(shù)及操作步驟詳見產(chǎn)品說明書。
  • 別名：
    CFHR3 ELISA kit; CFHL3 ELISA kit; FHR3Complement factor H-related protein 3 ELISA kit; FHR-3 ELISA kit; DOWN16 ELISA kit; H factor-like protein 3 ELISA kit
  • 縮寫：
    CFHR3
  • Uniprot No.：
  • 種屬：
    Homo sapiens (Human)
  • 樣本類型：
    serum, plasma, tissue homogenates
  • 檢測范圍：
    31.25 ng/mL-2000 ng/mL
  • 靈敏度：
    7.8 ng/mL
  • 反應(yīng)時間：
    1-5h
  • 樣本體積：
    50-100ul
  • 檢測波長：
    450 nm
  • 研究領(lǐng)域：
    Immunology
  • 測定原理：
    quantitative
  • 測定方法：
    Sandwich
  • 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 CFHR3 in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
    SampleSerum(n=4)
    1:100Average %95
    Range %90-99
    1:200Average %101
    Range %97-104
    1:400Average %96
    Range %89-99
    1:800Average %98
    Range %93-101
  • 回收率：
    The recovery of human CFHR3 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 TypeAverage % RecoveryRange
    Serum (n=5) 9185-94
    EDTA plasma (n=4)9390-97
  • 標(biāo)準(zhǔn)曲線：
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
    ng/mlOD1OD2AverageCorrected
    20002.623 2.632 2.628 2.532
    10001.814 1.788 1.801 1.705
    5000.988 0.963 0.976 0.880
    2500.571 0.554 0.563 0.467
    1250.334 0.347 0.341 0.245
    62.50.261 0.253 0.257 0.161
    31.250.153 0.166 0.160 0.064
    00.097 0.095 0.096
  • 數(shù)據(jù)處理：
  • 貨期：
    3-5 working days

產(chǎn)品評價

靶點(diǎn)詳情

  • 功能：
    Might be involved in complement regulation.
  • 基因功能參考文獻(xiàn)：
    1. 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
    2. 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
    3. These data provide evidence that FHR3, which is absent in patients with the autoimmune form of hemolytic uremic syndrome, is involved in B cell regulation PMID: 27279373
    4. Exploratory analyses of clinical and histopathologic parameters using the Oxford classification criteria revealed a suggestive association of CFHR3,1Delta with reduced tubulointerstitial injury. These data indicate that dysregulated activity of the alternative complement pathway contributes to IgAN pathogenesis in both Asians and Europeans and implicate CFHR3,1Delta as the functional allele at this locus PMID: 26940089
    5. 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
    6. 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.[CFHR3] PMID: 27196323
    7. 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
    8. Genetic variants in CFH, CFHR3, and CFHR1 affect complement activation and thereby predispose patients to develop IgA nephropathy. PMID: 25205734
    9. 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
    10. Atypical haemolytic-uraemic syndrome due to heterozygous mutations of CFH/CFHR1-3 and complement factor H 479. PMID: 24333077
    11. 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
    12. genetic variations in CFH and its related genes may contribute to hypertension risk in Chinese Hans PMID: 22848687
    13. A hybrid CFHR3-1 gene causes familial C3 glomerulopathy. PMID: 22626820
    14. Significant association was identified for the CFHR3-1 deletion in age-related macular degeneration cases, for both neovascular disease and geographic atrophy. PMID: 22558131
    15. A novel hybrid CFH/CFHR3 gene generated by a microhomology-mediated deletion is associated with familial atypical hemolytic uremic syndrome. PMID: 22058112
    16. Data show that 698 CNPs loci overlap with known disease-associated or pharmacogenetic-related genes such as CFHR3, CFHR1, GSTTI and UGT2B17. PMID: 21677662
    17. 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
    18. Combined deletion of CFHR3 and CFHR1is associated with a decreased risk of developing age-related macular degeneration. PMID: 21850184
    19. A change in gene dosage of the encoded proteins CFHR3 and CFHR1 might account for the increased systemic lupus erythematosus. PMID: 21637784
    20. 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
    21. Data from haplotype analysis demonstrates the relationship between the CFH rs10737680 association and the CFHR1-3Delta association in age-related macular degeneration. PMID: 20581873
    22. 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
    23. 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
    24. 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
    25. Results describe a correlation between the development of complement factor H autoantibodies and the deficiency of the CFH-related proteins, CFHR1 and CFHR3, in atypical hemolytic uremic syndrome. PMID: 19531976
    26. Deletion of CFHR3 and CFHR1 protected against the development of AMD at least in part because the deletion tagged a protective haplotype and did not occur on the risk haplotype. PMID: 19553609
    27. A common haplotype was associated with decreased risk of AMD. This haplotype carried a deletion of CFHR1 and CFHR3, and the proteins encoded by these genes were absent in serum of homozygotes. PMID: 16998489
    28. First detailed description of structure of human FHR3 gene PMID: 10781834

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  • 亞細(xì)胞定位：
    Secreted.
  • 組織特異性：
    Expressed by the liver and secreted in plasma.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 16980

    OMIM: 605336

    KEGG: hsa:10878

    STRING: 9606.ENSP00000356395

    UniGene: Hs.709217