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  4. Mouse Phenylalanine-4-hydroxylase(PAH) ELISA kit

Mouse Phenylalanine-4-hydroxylase(PAH) ELISA kit

  • 中文名稱：
    小鼠苯丙氨酸羥化酶4(PAH)酶聯免疫試劑盒
  • 貨號：
    CSB-EL017396MO
  • 規格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 其他：

產品詳情

  • 產品描述：
    小鼠苯丙氨酸羥化酶4(PAH)酶聯免疫試劑盒（CSB-EL017396MO）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿、細胞裂解物樣本中的PAH含量。PAH（苯丙氨酸羥化酶）是參與苯丙氨酸代謝的關鍵酶。其基因突變會導致苯丙酮尿癥等疾病。研究機制主要聚焦于解析其結構與功能關系，探索恢復或增強酶活性的方法，以開發治療相關疾病的藥物和療法。試劑盒檢測范圍為23.5 pg/mL-1500 pg/mL，適用于實驗室環境下探究PAH在小鼠模型中的表達水平變化，如代謝通路研究、基因編輯動物模型的表型驗證、藥物干預對酶活性影響的評估等科研場景。可為細胞生物學、分子病理學及基礎醫學研究提供可靠的數據支持。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：
    Pah ELISA Kit; Phenylalanine-4-hydroxylase ELISA Kit; PAH ELISA Kit; EC 1.14.16.1 ELISA Kit; Phe-4-monooxygenase ELISA Kit
  • 縮寫：
  • Uniprot No.：
  • 種屬：
    Mus musculus (Mouse)
  • 樣本類型：
    serum, plasma, tissue homogenates, cell lysates
  • 檢測范圍：
    23.5 pg/mL-1500 pg/mL
  • 靈敏度：
    5.8 pg/mL
  • 反應時間：
    1-5h
  • 樣本體積：
    50-100ul
  • 檢測波長：
    450 nm
  • 研究領域：
    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 mouse PAH 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 % 104
    Range % 101-109
    1:2 Average % 95
    Range % 91-99
    1:4 Average % 86
    Range % 83-89
    1:8 Average % 92
    Range % 87-96
  • 回收率：
    The recovery of mouse PAH 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) 86 83-89
    EDTA plasma (n=4) 89 85-96
  • 標準曲線：
    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
    1500 2.646 2.701 2.674 2.481
    750 2.063 2.000 2.032 1.839
    375 1.324 1.405 1.365 1.172
    187.5 0.826 0.834 0.830 0.637
    94 0.593 0.587 0.590 0.397
    47 0.362 0.371 0.367 0.174
    23.5 0.278 0.284 0.281 0.088
    0 0.192 0.194 0.193 　
  • 數據處理：
  • 貨期：
    3-5 working days

產品評價

相關問答

■ 常見問題解答
Q:

Our customer would like to know if you have cross-reactivity data for the CSB-EL017396MO PAH ELISA kit (Mouse).
They are curious to know whether you have tested to see if this mouse kit cross-reacts with human.
Thanks for your assistance.

A:
Thanks for your inquiry!
For different species, the primary structure and the advanced structure of proteins are different. The antibody's characteristics are also different. So we didn't validate human samples with mouse kit before. We don't suggest you use this kit to test human samples. Sorry we don't have human pah ELisa kit.
Q:

What is the contents of the antibody in this kit?

A:
Thanks for your inquiry!
Capture antibody is goat polyclonal antibody and detection antibody is goat polyclonal antibody.

靶點詳情

  • 功能：
    Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine.
  • 基因功能參考文獻：
    1. We propose that CNS monoamine deficiency may be the cause of the partially reversible adverse behavioral effects associated with chronic HPA in Pah(enu2) mice, but that phenylalanine-reducing treatments initiated during adulthood are unable to correct the neuropathology and attendant cognitive deficits that develop during juvenile life in late-treated Pah(enu2/enu2) mice PMID: 29331172
    2. we assessed DNA methylation patterns in brain tissues using methylated DNA immunoprecipitation and paired end sequencing in adult PAH(enu2) animals maintained under either continuous dietary Phe restriction or chronic hyperphenylalaninemia. Heterozygous PAH(enu2/WT) litter mates served as controls for normal Phe exposure PMID: 26822703
    3. These observations support the proposition that differences in phenylalanine hydroxylase activity underlie the variation in S-carboxymethyl-L-cysteine sulfoxidation and that no other enzyme is able to undertake this reaction. PMID: 26338263
    4. A single injection of PAH-RBCs was able to decrease Phe levels by nearly 80% in normal mice PMID: 23867524
    5. high levels of Phe, such as in phenylketonuria, are unlikely to directly induce brain hypomyelination, suggesting involvement of alternative mechanisms in this myelination defect PMID: 20151197
    6. phenylalanine concentration regulates the activity of PAH in the hph-1 mouse and that this acts via a mechanism that includes phosphorylation of the PAH molecule PMID: 19560382
    7. Tetrahydrobiopterin protects PAH activity in vivo. PMID: 15556637
    8. low level of phenylalanine clearance was observed in mice expressing PAH and GTP-CH from the keratin 14 promoter PMID: 15946242

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  • 相關疾?。?/div>
    Mouse strains deficient in phenylalanine hydroxylase (Pah) were created as models of phenylketonuria (PKU).
  • 蛋白家族：
    Biopterin-dependent aromatic amino acid hydroxylase family
  • 數據庫鏈接：


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