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  4. Mouse β-hexosaminidase A,β-Hex A ELISA Kit

Mouse β-hexosaminidase A,β-Hex A ELISA Kit

  • 中文名稱：
    小鼠β氨基己糖苷酶A(β-Hex A)酶聯(lián)免疫試劑盒
  • 貨號：
    CSB-E04818m
  • 規(guī)格：
    96T/48T
  • 價格：
    ￥3800/￥2500
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品描述：
    小鼠β氨基己糖苷酶A(β-Hex A)酶聯(lián)免疫試劑盒（CSB-E04818m）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HEXA含量。HEXA是一種溶酶體酶，負責(zé)分解GM2神經(jīng)節(jié)苷脂，維持細胞內(nèi)環(huán)境穩(wěn)定。其突變會導(dǎo)致GM2神經(jīng)節(jié)苷脂在細胞內(nèi)積累，引發(fā)泰薩二氏病等疾病。研究HEXA的機制，旨在開發(fā)治療相關(guān)遺傳性神經(jīng)退行性疾病的藥物。試劑盒檢測范圍為3.12 μU/mL-200 μU/mL，適用于研究β-Hex A在代謝性疾病模型小鼠中的表達調(diào)控、基因敲除/過表達動物模型的酶活性驗證，以及藥物干預(yù)或病理條件下溶酶體功能的評估等科研場景。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：
    Hexa ELISA Kit; Beta-hexosaminidase subunit alpha ELISA Kit; EC 3.2.1.52 ELISA Kit; Beta-N-acetylhexosaminidase subunit alpha ELISA Kit; Hexosaminidase subunit A ELISA Kit; N-acetyl-beta-glucosaminidase subunit alpha ELISA Kit
  • 縮寫：
  • Uniprot No.：
  • 種屬：
    Mus musculus (Mouse)
  • 樣本類型：
    serum, plasma, tissue homogenates
  • 檢測范圍：
    3.12 μU/mL-200 μU/mL
  • 靈敏度：
    0.78 μU/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：
    Neuroscience
  • 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 β-Hex A in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
    SampleSerum(n=4)
    1:10Average %92
    Range %85-100
    1:20Average %93
    Range %87-98
    1:40Average %90
    Range %86-100
    1:80Average %90
    Range %86-98
  • 回收率：
    The recovery of mouse β-Hex A 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) 9789-101
    EDTA plasma (n=4)9592-98
  • 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.
    μU/mlOD1OD2AverageCorrected
    2002.542 2.517 2.530 2.386
    1002.073 2.101 2.087 1.943
    501.452 1.464 1.458 1.314
    250.942 0.961 0.952 0.808
    12.50.416 0.402 0.409 0.265
    6.250.325 0.311 0.318 0.174
    3.120.247 0.237 0.242 0.098
    00.146 0.141 0.144
  • 數(shù)據(jù)處理：
  • 貨期：
    3-5 working days

產(chǎn)品評價

靶點詳情

  • 功能：
    Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides. The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6-sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2. The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide. Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A.
  • 基因功能參考文獻：
    1. Neu4 is not the only sialidase contributing to the metabolic bypass in Hexa(-/-) mice. PMID: 20862357
  • 亞細胞定位：
    Lysosome.
  • 蛋白家族：
    Glycosyl hydrolase 20 family
  • 組織特異性：
    Ubiquitous. Most abundant in testis, adrenal, epididymis and heart. Low levels seen in the liver.
  • 數(shù)據(jù)庫鏈接：

    KEGG: mmu:15211

    STRING: 10090.ENSMUSP00000026262

    UniGene: Mm.2284