Human complement factor I(CFI) ELISA Kit
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中文名稱:人補體因子I(CFI)酶聯(lián)免疫試劑盒
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貨號:CSB-E13108h
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規(guī)格:96T/48T
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價格:¥3600/¥2500
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其他:
產(chǎn)品詳情
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產(chǎn)品描述:人補體因子I(CFI)酶聯(lián)免疫試劑盒(CSB-E13108h)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CFI含量。試劑盒檢測范圍為6.25 ng/mL-400 ng/mL,本試劑盒基于高特異性抗體對目標(biāo)蛋白的捕獲與檢測,實驗流程標(biāo)準(zhǔn)化,可滿足科研場景下對CFI表達的定量需求,例如探究補體系統(tǒng)相關(guān)疾病的分子機制、評估炎癥或免疫紊亂模型中CFI的動態(tài)變化,或用于組織微環(huán)境中補體調(diào)控因子的基礎(chǔ)研究。試劑盒支持多種生物樣本類型,為體外實驗提供靈活選擇,適用于細胞生物學(xué)、免疫學(xué)及病理生理學(xué)等領(lǐng)域的定量分析需求。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。
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別名:AHUS3 ELISA Kit; ARMD13 ELISA Kit; C3b INA ELISA Kit; C3b inactivator ELISA Kit; C3B/C4B inactivator ELISA Kit; C3BINA ELISA Kit; CFAI_HUMAN ELISA Kit; Cfi ELISA Kit; Complement component I ELISA Kit; Complement control protein factor I ELISA Kit; Complement factor I ELISA Kit; Complement factor I heavy chain ELISA Kit; Complement factor I light chain ELISA Kit; F1 ELISA Kit; factor I ELISA Kit; FactorI ELISA Kit; FI ELISA Kit; I factor ELISA Kit; IF ELISA Kit; KAF ELISA Kit; Konglutinogen activating factor ELISA Kit; Light chain of factor I ELISA Kit; OTTHUMP00000219728 ELISA Kit; OTTHUMP00000221928 ELISA Kit
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縮寫:
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Uniprot No.:
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種屬:Homo sapiens (Human)
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樣本類型:serum, plasma, tissue homogenates
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檢測范圍:6.25 ng/mL-400 ng/mL
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靈敏度:1.56 ng/mL
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反應(yīng)時間:1-5h
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樣本體積:50-100ul
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檢測波長:450 nm
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研究領(lǐng)域:Immunology
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測定原理:quantitative
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測定方法:Sandwich
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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 CFI 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) 1:200 Average % 92 Range % 89-94 1:400 Average % 101 Range % 98-103 1:800 Average % 92 Range % 89-96 1:1600 Average % 97 Range % 94-102 -
回收率:
The recovery of human CFI 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) 95 89-98 EDTA plasma (n=4) 96 92-98 -
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/ml OD1 OD2 Average Corrected 400 2.780 2.766 2.773 2.635 200 2.288 2.311 2.300 2.162 100 1.743 1.684 1.714 1.576 50 1.114 1.101 1.108 0.970 25 0.622 0.652 0.637 0.499 12.5 0.345 0.336 0.341 0.203 6.25 0.237 0.224 0.231 0.093 0 0.140 0.136 0.138 -
數(shù)據(jù)處理:
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貨期:3-5 working days
相關(guān)產(chǎn)品
靶點詳情
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功能:Trypsin-like serine protease that plays an essential role in regulating the immune response by controlling all complement pathways. Inhibits these pathways by cleaving three peptide bonds in the alpha-chain of C3b and two bonds in the alpha-chain of C4b thereby inactivating these proteins. Essential cofactors for these reactions include factor H and C4BP in the fluid phase and membrane cofactor protein/CD46 and CR1 on cell surfaces. The presence of these cofactors on healthy cells allows degradation of deposited C3b by CFI in order to prevent undesired complement activation, while in apoptotic cells or microbes, the absence of such cofactors leads to C3b-mediated complement activation and subsequent opsonization.
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基因功能參考文獻:
- This study has revealed a significant genetic role for CFI-rs13104777 in acute anterior uveitis. This influence may be dependent on human leukocyte antigen (HLA)-B27 and disease laterality. PMID: 27380463
- An extremely rare, heterozygous mutation in the gene encoding CFI likely affecting splicing was associated for the first time with atypical hemolytic uremic syndrome. PMID: 28455885
- this study illustrates the importance of early versus late diagnosis of CFI deficiency PMID: 28942469
- This finding although rare does suggest that screening for chromosomal rearrangements affecting CFI should be undertaken in all aHUS patients particularly if the factor I level is unexplainably low. PMID: 27268256
- 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
- Taken together, our data argue that multiple rare and ultra-rare alleles in CFI contribute to AMD pathogenesis; they improve the precision of the assessment of the contribution of CFI to AMD PMID: 28282489
- Case Report: thrombotic microangiopathy with mutations in complement factor I and thrombomodulin. PMID: 26613809
- Our results indicate that CFI polymorphisms are not significantly associated with VKH syndrome. PMID: 26900322
- Patients with advanced atrophic AMD carried these rare variants more frequently than patients with neovascular AMD (11 of 93 [11.8%] vs 40 of 835 [4.8%]; P = .04). PMID: 26767664
- Low FI levels are strongly associated with rare CFI variants and age-related macular degeneration. PMID: 25788521
- A missense variant (p.V412M) in CFI was discovered in two Tunisian Jewish families with early-onset age-related macular degeneration. PMID: 25986072
- Regulatory components of the alternative complement pathway in endothelial cell cytoplasm, factor H and factor I, are not packaged in Weibel-Palade bodies. PMID: 25803806
- In this study, the odds of AMD were highest in those with deficient vitamin D status and 2 risk alleles for the CFH and CFI genotypes, suggesting a synergistic effect between vitamin D status and complement cascade protein function. PMID: 26312598
- iC3b level, a proteolytically inactive form of C3b, was lower in HCV infected patient sera, reflecting impairment of both C3 convertase and Factor I activity. PMID: 24983375
- association between rs10033900 and age-related macular degeneration risk in Han Chinese population PMID: 24642830
- The mutations in the regulators CFH, CFI and MCP involve loss-of-function, whereas those for C3 involve gain-of-function. PMID: 25188723
- The CFI p.Gly119Arg mutation was identified in 7/521 age-related macular degeneration cases compared to 1/627 age-matched controls; this mutation confers a high risk of disease. PMID: 25352734
- CFI genetic variants played an important role in the development of NSCLC in Chinese population. PMID: 25394898
- High expression of complement factor I is associated with recurrence in breast cancer. PMID: 25618258
- results provide evidence for the role of CFI in the progression of cSCC and identify it as a potential therapeutic target in this nonmelanoma skin cancer PMID: 25184960
- This study has revealed a significant association between acute anterior uveitis (AAU) and CFI-rs7356506, suggesting that CFI is involved in the pathogenesis of AAU PMID: 25075123
- Neither of the two SNPs most studied (rs10033900 or rs2285714) in the CFI gene was a risk factor for developing neovascular age-related macular degeneration or polypoidal choroidal vasculopathy in a Chinese population. PMID: 24732209
- Recurrent aseptic meningo-encephalitis is a rare clinical presentation of complete FI deficiency. PMID: 24142231
- An STR polymorphism in intron 7 of human CFI gene on chromosome 4q in 11 Asian populations indicated that Group H alleles in exon 11 of the CFI gene were almost entirely confined to East Asian populations, making it useful in forensic anthropology. PMID: 23688582
- We found that 7.8% of advanced age-related macular degeneration cases compared to 2.3% of controls are carriers of rare missense CFI variants. PMID: 24036952
- Case Report: patient with atypical haemolytic uremic syndrome with combined membrane cofactor protein CD46 and complement factor I mutations undergoing successful kidney transplantation. PMID: 23519521
- Mutations in complement factor I protein is associated with end-stage renal disease in a patient with hemolytic uremic syndrome caused by infections by Escherichia coli strains producing Shiga-like toxins. PMID: 23731345
- these findings demonstrate that rare, highly penetrant mutations in CFI contribute to the genetic burden of age-related macular degeneration. PMID: 23685748
- rs1136287 in CFI is less likely to be associated in in extremely myopic Japanese individuals. PMID: 23722394
- The alternative pathway of complement may play a role in the pathogenesis of HELLP syndrome. PMID: 22594569
- Acute hemorrhagic leukoencephalitis (AHLE) is an unreported, rare phenotype for partial complement factor I deficiency. PMID: 22926405
- we report four novel mutations and the first large gene deletion in the CFI locus. PMID: 22710145
- One SNP (rs10033900) in the CFI gene, which encodes a protein involved in the inflammatory pathway, was significantly associated with myopic choroidal neovascularization in multivariate analysis after correction for multiple testing. PMID: 22678500
- Since FI requires cofactors for its activity we also investigated the binding of purified cofactors C4BP and FH and found acquisition of both proteins, which retained their activity in FI mediated degradation of C3b and C4b. PMID: 22514678
- factor I were significantly diminished early after trauma. PMID: 22258234
- all analyzed cofactors form similar trimolecular complexes with FI and C3b/C4b, and the accessibility of FIMAC and SP domains is crucial for the function of FI PMID: 22393059
- Results question whether complement factor I autoantibodies per se predispose to atypical hemolytic uremic syndrome. PMID: 22223611
- Forster resonance energy transfer was used to investigate the 10 muM K(D) (210 kD) complex between the N-terminal region of the soluble complement regulator, factor H (FH1-4), and the key activation-specific complement fragment, C3b. PMID: 21936007
- Data show that FI is in a proteolytically inactive form, demonstrating that it circulates in a zymogen-like state. PMID: 21768352
- Study describes the molecular and functional consequences of two novel mutations of FI. PMID: 21316765
- Study identified novel mutations in CFH, CFHR5, CFI, CFB and C3 in American patients with atypical hemolytic uremic syndrome. PMID: 20513133
- Role of a common variant near the complement factor I gene in susceptibility to age-related macular degeneration. PMID: 20087399
- In a large cohort of 202 patients with aHUS, we identified 23 patients carrying exonic mutations in CFI PMID: 20016463
- the FIMAC domain appears to harbor the main binding sites important for the ability of FI to degrade C4b and C3b PMID: 20044478
- mutations in complement factor I affect both secretion and function of factor I, which leads to impaired regulation of the complement system in atypical hemolytic uremic syndrome. PMID: 19877009
- the last 45 amino acid of the heavy chain, including a disulfide bridge area, did not participate in the serine protease function of factor I PMID: 14967308
- Human complement factor I does not require cofactors for cleavage of synthetic substrates. PMID: 15210795
- fI and the serine protease domain were found to have similar amidolytic activities but strikingly different proteolytic activities on C3(NH(3)). PMID: 15835912
- Mutations in the complement regulators factor H, membrane cofactor protein (MCP), and factor I are associated with atypical hemolytic uremic syndrome. PMID: 16386793
- factor I in concert with CR1 on E and factor H in serum due to their cofactor activity are likely to be important contributors PMID: 16920989
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相關(guān)疾病:Hemolytic uremic syndrome atypical 3 (AHUS3); Complement factor I deficiency (CFI deficiency); Macular degeneration, age-related, 13 (ARMD13)
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亞細胞定位:Secreted, extracellular space. Secreted.
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蛋白家族:Peptidase S1 family
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組織特異性:Expressed in the liver by hepatocytes. Also present in other cells such as monocytes, fibroblasts or keratinocytes.
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