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  4. Human NADH-cytochrome b5 reductase 3(CYB5R3) ELISA kit

Human NADH-cytochrome b5 reductase 3(CYB5R3) ELISA kit

  • 中文名稱：
    人NADH-細(xì)胞色素b5還原酶3(CYB5R3)酶聯(lián)免疫試劑盒
  • 貨號：
    CSB-EL006320HU
  • 規(guī)格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品描述：
    人NADH-細(xì)胞色素b5還原酶3(CYB5R3)酶聯(lián)免疫試劑盒（CSB-EL006320HU）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿、細(xì)胞裂解物樣本中的CYB5R3含量。CYB5R3是重要靶點。它參與體內(nèi)多種生物化學(xué)反應(yīng)，如脂肪酸代謝等。研究機(jī)制方面，主要聚焦其在電子傳遞鏈中的作用，明確其對維持細(xì)胞內(nèi)環(huán)境穩(wěn)定意義重大。其功能異常和一些疾病相關(guān)，目前正深入研究為疾病治療提供方向。試劑盒檢測范圍為25 pg/mL-1600 pg/mL，適用于研究CYB5R3在氧化應(yīng)激反應(yīng)、脂質(zhì)代謝調(diào)控中的分子機(jī)制，或作為藥物開發(fā)中代謝通路研究的檢測工具，亦可用于遺傳代謝疾病模型構(gòu)建時的生物標(biāo)志物分析。為血紅素代謝通路研究、藥物毒性評估及疾病機(jī)制探索提供靈敏的定量檢測方案，滿足細(xì)胞生物學(xué)、藥理學(xué)等領(lǐng)域的科研需求。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。
  • 別名：
    B5R ELISA Kit; Cyb5r3 ELISA Kit; Cytochrome b5 reductase 3 ELISA Kit; Cytochrome b5 reductase ELISA Kit; DIA1 ELISA Kit; Diaphorase 1 ELISA Kit; Diaphorase-1 ELISA Kit; NADH cytochrome b5 reductase 3 ELISA Kit; NADH-cytochrome b5 reductase 3 membrane-bound form ELISA Kit; NADH-cytochrome b5 reductase 3 soluble form ELISA Kit; NB5R3_HUMAN ELISA Kit; OTTHUMP00000028761 ELISA Kit; OTTHUMP00000198435 ELISA Kit; OTTHUMP00000198574 ELISA Kit; OTTHUMP00000198662 ELISA Kit; OTTHUMP00000198665 ELISA Kit
  • 縮寫：
  • Uniprot No.：
  • 種屬：
    Homo sapiens (Human)
  • 樣本類型：
    serum, plasma, tissue homogenates, cell lysates
  • 檢測范圍：
    25 pg/mL-1600 pg/mL
  • 靈敏度：
    6.25 pg/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：
    Metabolism
  • 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 CYB5R3 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)
    1:1 Average % 105
    Range % 94-110
    1:2 Average % 104
    Range % 97-109
    1:4 Average % 94
    Range % 83-99
    1:8 Average % 90
    Range % 83-93
  • 回收率：
    The recovery of human CYB5R3 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 84-96
    EDTA plasma (n=4) 107 100-111
  • 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.
    pg/ml OD1 OD2 Average Corrected
    1600 2.323 2.263 2.293 2.183
    800 1.723 1.649 1.686 1.576
    400 1.208 1.158 1.183 1.073
    200 0.642 0.666 0.654 0.544
    100 0.423 0.417 0.420 0.310
    50 0.270 0.264 0.267 0.157
    25 0.185 0.179 0.182 0.072
    0 0.108 0.112 0.110 　
  • 數(shù)據(jù)處理：
  • 貨期：
    3-5 working days

產(chǎn)品評價

靶點詳情

  • 功能：
    Desaturation and elongation of fatty acids, cholesterol biosynthesis, drug metabolism, and, in erythrocyte, methemoglobin reduction.
  • 基因功能參考文獻(xiàn)：
    1. study indicated that novel homozygous mutation p.Arg192Cys in CYB5R3 gene present in eight cases and the possibility of high prevalence of heterozygous in Indian population causing Type I recessive congenital methemoglobinemia. PMID: 29482478
    2. CYB5R3 promotes colonization and metastasis formation and is a prognostic marker of disease-free and overall survival in estrogen receptor-negative breast cancer. PMID: 26351264
    3. Genetic variation in CYB5R3 is associated with methemoglobin levels in preterm infants receiving nitric oxide therapy. PMID: 25521918
    4. The results unveil a potential mechanism of action by which CYB5R3 deficiency contributes to the pathophysiological underpinnings of neurological disorders in RHM patients. PMID: 24450884
    5. NADH-CYB5R deficiency causes two forms of recessive congenital methemoglobinemia with cyanosis. PMID: 24266649
    6. Dapsone-associated methemoglobinemia in a patient with slow NAT2*5B haplotype and impaired cytochrome b5 reductase activity PMID: 21422237
    7. A comprehensive overview of the study of structure and function of human cytochrome b5 reductase. PMID: 23113554
    8. Data indicate that mitochondrial amidoxime reducing components 1 and 2 together with the electron transport proteins NADH-cytochrome b5 reductase (CYB5R) and cytochrome b5 (CYB5) catalyze the reduction of N-hydroxylated compounds such as amidoximes. PMID: 23703616
    9. Population frequency and age of c.806C > T mutation in CYB5R3 gene as cause of recessive congenital methemoglobinemia in Yakutia. PMID: 23866629
    10. Novel large deletion c.22-1320_633+1224del in the CYB5R3 gene from patients with hereditary methemoglobinemia PMID: 23297489
    11. CYB5R3 gene of three probands with type I methemoglobinemia and their relatives were sequenced revealing several putative causative mutations; in one subject multiple mutations were present PMID: 21349748
    12. We conclude that Cytochrome b(5)and cytochrome b(5) reductase catalyze the reduction of arylhydroxylamines in breast tissue. PMID: 21447608
    13. novel allelic mutation identified at codon 235 is in helix 5; first report of mental retardation because of the novel mutation, along with a second mutation in the NADH-b5R gene in an Indian family with recessive congenital methemoglobinemia Type II PMID: 21328435
    14. Dia1 is localized to the perinuclear endoplasmic reticulum in an RNA-zipcode-independent manner in fibroblasts. PMID: 21266463
    15. It was shown that Yakut patients have none of three missence mutations, Arg57Gln, Leu72Pro, and Val105Met, described in case of this disease in the neighboring populations, Chinese and Japanese, inhabiting the territories south of Yakutia PMID: 12884529
    16. A decrease of the activity of membrane-bound NADH-methemoglobin reductase and a change of physical state of the lipid bilayer of membranes under oxidative stress were found in erythrocytes in vivo and in vitro. PMID: 15039026
    17. Amino acid substitution results in congsenital methemoglobinemia. PMID: 15297856
    18. crystal structure of cytochrome b(5) reductase PMID: 15502298
    19. Recessive congenital methaemoglobinaemia observed in a Lebanese subject with a novel mutation in NADH-cytochrome b5 reductase gene. PMID: 15813912
    20. A novel intronic mutation at 22163 caused markedly reduced mRNA (7% of normal) resulting in type II methemoglobinemia. PMID: 15921385
    21. Dia1 is required for the formation of the actin coat around endosomes downstream of RhoB, connecting membrane trafficking with the regulation of actin dynamics. PMID: 15944396
    22. DIA1 and IQGAP1 interact in cell migration and phagocytic cup formation. PMID: 17620407
    23. report of the clinical and molecular characteristics of 6 new patients with recessive hereditary methemoglobinemia due to cytochrome b5 reductase deficiency; two new mutations of DIA1, c. 82 C>T(Gln27STOP) and c. 136 C>T(Arg45Trp), were found PMID: 18343696
    24. The decline in the activities of G6PD and b5Rm would indicate a decrease in the antioxidant response associated with RBC aging. PMID: 19811411

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  • 相關(guān)疾?。?/div>
    Methemoglobinemia CYB5R3-related (METHB-CYB5R3)
  • 亞細(xì)胞定位：
    [Isoform 1]: Endoplasmic reticulum membrane; Lipid-anchor; Cytoplasmic side. Mitochondrion outer membrane; Lipid-anchor; Cytoplasmic side.; [Isoform 2]: Cytoplasm. Note=Produces the soluble form found in erythrocytes.
  • 蛋白家族：
    Flavoprotein pyridine nucleotide cytochrome reductase family
  • 組織特異性：
    Isoform 2 is expressed at late stages of erythroid maturation.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 2873

    OMIM: 250800

    KEGG: hsa:1727

    STRING: 9606.ENSP00000354468

    UniGene: Hs.561064