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  4. Recombinant Human Beta-hexosaminidase subunit beta (HEXB)

Recombinant Human Beta-hexosaminidase subunit beta (HEXB)

  • 货号：
    CSB-YP010316HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 货号：
    CSB-EP010316HU-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 货号：
    CSB-BP010316HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 货号：
    CSB-MP010316HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 别名：
    Beta hexosaminidase beta chain; Beta hexosaminidase subunit beta; Beta N acetylhexosaminidase; Beta-hexosaminidase subunit beta chain A; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto oncogene 7 protein; Cervical cancer proto-oncogene 7 protein; ENC 1AS; Epididymis luminal protein 248; HCC 7; HCC-7; HCC7; HEL 248; HEX B; Hexb; HEXB_HUMAN; Hexosaminidase B (beta polypeptide); Hexosaminidase B; Hexosaminidase subunit B; HexosaminidaseB; N acetyl beta glucosaminidase; N-acetyl-beta-glucosaminidase subunit beta
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length of Mature Protein
  • 表达区域：
    122-556
  • 氨基酸序列
    TQVQQLLVS ITLQSECDAF PNISSDESYT LLVKEPVAVL KANRVWGALR GLETFSQLVY QDSYGTFTIN ESTIIDSPRF SHRGILIDTS RHYLPVKIIL KTLDAMAFNK FNVLHWHIVD DQSFPYQSIT FPELSNKGSY SLSHVYTPND VRMVIEYARL RGIRVLPEFD TPGHTLSWGK GQKDLLTPCY SRQNKLDSFG PINPTLNTTY SFLTTFFKEI SEVFPDQFIH LGGDEVEFKC WESNPKIQDF MRQKGFGTDF KKLESFYIQK VLDIIATINK GSIVWQEVFD DKAKLAPGTI VEVWKDSAYP EELSRVTASG FPVILSAPWY LDLISYGQDW RKYYKVEPLD FGGTQKQKQL FIGGEACLWG EYVDATNLTP RLWPRASAVG ERLWSSKDVR DMDDAYDRLT RHRCRMVERG IAAQPLYAGY CNHENM
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

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