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  4. Human N-acetyl-β-D-glucosaminidase,NAG ELISA Kit

Human N-acetyl-β-D-glucosaminidase,NAG ELISA Kit

  • 中文名称：
    人N-乙酰-β-D-氨基葡萄糖苷酶(NAG)酶联免疫试剂盒
  • 货号：
    CSB-E09450h
  • 规格：
    96T/48T
  • 价格：
    ￥3600/￥2500
  • 其他：

产品详情

  • 产品描述：

    This Human NAG ELISA Kit was designed for the quantitative measurement of Human NAG protein in serum, urine. It is a Competitive ELISA kit, its detection range is 7.81 mIU/mL-2000 mIU/mL and the sensitivity is 7.81 mIU/mL.

  • 别名：
    Beta hexosaminidase beta chain ELISA Kit; Beta hexosaminidase subunit beta ELISA Kit; Beta N acetylhexosaminidase ELISA Kit; Beta-hexosaminidase subunit beta chain A ELISA Kit; Beta-N-acetylhexosaminidase subunit beta ELISA Kit; Cervical cancer proto oncogene 7 protein ELISA Kit; Cervical cancer proto-oncogene 7 protein ELISA Kit; ENC 1AS ELISA Kit; Epididymis luminal protein 248 ELISA Kit; HCC 7 ELISA Kit; HCC-7 ELISA Kit; HCC7 ELISA Kit; HEL 248 ELISA Kit; HEX B ELISA Kit; Hexb ELISA Kit; HEXB_HUMAN ELISA Kit; Hexosaminidase B (beta polypeptide) ELISA Kit; Hexosaminidase B ELISA Kit; Hexosaminidase subunit B ELISA Kit; HexosaminidaseB ELISA Kit; N acetyl beta glucosaminidase ELISA Kit; N-acetyl-beta-glucosaminidase subunit beta ELISA Kit
  • 缩写：
    NAG
  • Uniprot No.：
  • 种属：
    Homo sapiens (Human)
  • 样本类型：
    serum, urine
  • 检测范围：
    7.81 mIU/mL-2000 mIU/mL
  • 灵敏度：
    7.81 mIU/mL
  • 反应时间：
    1-5h
  • 样本体积：
    50-100ul
  • 检测波长：
    450 nm
  • 研究领域：
    Others
  • 测定原理：
    quantitative
  • 测定方法：
    Competitive
  • 本试剂盒所含材料：
    • A 96-well Assay plate --The 96-well plate has been pre-coated with NAG.
    • Standard (2 x 250 μl)--Dilute the standard at dilution series, read the OD values, and then draw a standard curve.
    • Anti-human NAG Antibody (100 x concentrate) (1 x 60 μl)
    • Antibody Diluent (1 x 10 ml) --Dilute the anti-human NAG antibody.
    • HRP-conjugated goat-anti-mouse NAG antibody (100 x concentrate) (1 x 120 μl) --Act as the detection antibody.
    • HRP-conjugate Diluent (1 x 20 ml) --dilute the HRP-conjugated NAG antibody.
    • Sample Diluent (2 x 20 ml) --Reconstitute the standard and dilute the sample to an appropriate concentration.
    • Wash Buffer (25x concentrate) (1 x 20 ml)--Wash away unbound or free substances.
    • TMB Substrate (1x 10 ml) --Act as the chromogenic agent. TMB interacts with HRP, eliciting the solution turns blue.
    • Stop Solution (1 x 10ml) --Stop the color reaction. The solution color immediately turns from blue to yellow.
    • Four Adhesive Strips (For 96 wells)
    • An Instruction manual

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  • 本试剂盒不含材料：
    • A microplate reader capable of measuring absorbance at 450 nm, with the correction wavelength set at 540 nm - 570 nm.
    • An incubator that can provide stable incubation conditions up to 37°C±5°C.
    • Centrifuge
    • Vortex
    • Squirt bottle, manifold dispenser, or automated microplate washer
    • Absorbent paper for blotting the microtiter plate
    • 50-300ul multi-channel micropipette
    • Pipette tips
    • Single-channel micropipette with different ranges
    • 100ml and 500ml graduated cylinders
    • Deionized or distilled water
    • Timer
    • Test tubes for dilution

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  • 数据处理：
  • 货期：
    3-5 working days

产品评价

靶点详情

  • 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 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. During fertilization is responsible, at least in part, for the zona block to polyspermy. Present in the cortical granules of non-activated oocytes, is exocytosed during the cortical reaction in response to oocyte activation and inactivates the sperm galactosyltransferase-binding site, accounting for the block in sperm binding to the zona pellucida.
  • 基因功能参考文献：
    1. a modified human hexosaminidase subunit beta (HexB), which we have termed mod2B, composed of homodimeric beta subunits that contain amino acid sequences from the alpha subunit that confer GM2 ganglioside-degrading activity and protease resistance. PMID: 27018595
    2. Mutations of the HEXB gene is associated with maple syrup urine disease or Sandhoff disease. PMID: 27682710
    3. report on the heterogeneity of the mutational spectrum of the HEXB gene in Indian patients with Sandhoff disease PMID: 26582265
    4. The absence of beta-N-acetyl-hexosaminidase activity does not alter the differentiation of i-DCs from HSCs, but it is critical for the activation of CD4(+)T cells because knock-down of HEXA or HEXB gene causes a loss of function of i-DCs. PMID: 21997228
    5. Concentration and specific activity of N-acetyl-B-hexosaminidase in palatine tonsils in patients with tonsillar hypertrophy and chronic tonsillitis both in childhood and adulthood significantly increase in comparison to healthy individuals. PMID: 23911049
    6. A total of 19 HEXB variants were found in the 1092 genomes of which 5 are suspected of having a deleterious effect on hexosaminidase activity. PMID: 24461908
    7. DNA from Iranian Tay-Sachs patients reveals a novel mutation in HEXB predicting a termination codon or nonsense mutation. PMID: 24518553
    8. A patient with Sandhoff disease also is found to have a compound macro-deletion in HEXB. PMID: 23886397
    9. A highly significant correlation of HEX-7 and %CDT has been found. Because of exclusion of the P isoform, HEX-7 could be a useful supplementary marker for detecting chronic alcohol abuse. PMID: 23906468
    10. Expression of beta-hexosaminidase in the neurons of Sandhoff disease patients rescues transgenic mice from neurodegeneration. PMID: 22863301
    11. minigene studies revealed the presence of a novel alternative spliced HEXB mRNA variant also present in normal cells PMID: 22848519
    12. Characterization of seven novel mutations on the HEXB gene in French Sandhoff patients. PMID: 23046579
    13. We describe a novel HEXB mutation that is shared among 4 patients with Sandhoff disease. PMID: 22191674
    14. Plasma beta-hexosaminidase and beta-galactosidase) levels are higher in patients with Alzheimer's disease-type 2 diabetes mellitus (T2DM) compared to those with T2DM alone. PMID: 21321400
    15. The X-ray crystal structure of beta-hexosaminidase B provides new insights into mutations that cause Sandhoff disease. PMID: 12706724
    16. novel c.1556A>G transition in exon 12 of the HEXB gene associated with chronic Sandhoff's disease PMID: 17251047
    17. Beta-hexosaminidase is a peptidoglycan hydrolase that surprisingly exerts its mycobactericidal effect at the macrophage plasma membrane during mycobacteria-induced secretion of lysosomes PMID: 18180457
    18. Elevated activity of beta-hexosaminidase observed in subjects with asthma suggests that the beta-hex isozyme could take part in airway inflammation and remodeling in asthma. PMID: 18204279
    19. Lysosome-related genes, such as CLN2, CLN3, and HEXB, may be involved in the pathogenesis of adipose tissue hypertrophy in TED. PMID: 18552385
    20. These results reveal a new aspect of beta-hexosaminidase biology and suggest that a fully processed membrane-associated form of Hex is translocated from the lysosomal membrane to the PM by an as yet unknown mechanism. W PMID: 18588514
    21. Results describe the molecular genetics of Sandhoff disease in Italy and provide new insights into the molecular basis of the disease through HEXB mutation. PMID: 18758829
    22. A new D459A missense HEXB mutation was discovered in six juvenile patients with Sandhoff disease. PMID: 18930675
    23. Gene therapy reduced GM(2) storage and ameliorated neuroinflammation in the brain of HexB(-/-) mice, as well as attenuated behavioral deficits. PMID: 19278737
    24. Data suggest that cigarette smoking can inhibit, by the influence on N-acetyl-beta-hexosaminidase activity, catabolism of oligosaccharide chains in cancer tissues. PMID: 19615986

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  • 相关疾病：
    GM2-gangliosidosis 2 (GM2G2)
  • 亚细胞定位：
    Lysosome. Cytoplasmic vesicle, secretory vesicle, Cortical granule.
  • 蛋白家族：
    Glycosyl hydrolase 20 family
  • 数据库链接：

    HGNC: 4879

    OMIM: 268800

    KEGG: hsa:3074

    STRING: 9606.ENSP00000261416

    UniGene: Hs.69293