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HEXB Antibody

  • 货号：
    CSB-PA224713
  • 规格：
    ￥2024
  • 图片：
    • Western blot analysis of extracts from Jurkat cells, using HEXB antibody.
    • Immunohistochemistry analysis of paraffin-embedded human colon carcinoma tissue using HEXB antibody.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) HEXB Polyclonal antibody
  • Uniprot No.：
    P07686
  • 基因名：
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthesized peptide derived from C-terminal of Human HEXB.
  • 免疫原种属：
    Homo sapiens (Human)
  • 克隆类型：
    Polyclonal
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:3000
    IHC 1:50-1:100
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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