在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

  1. 首頁
  2. 蛋白
  3. 重組蛋白
  4. Recombinant Human Beta-hexosaminidase subunit beta (HEXB)

Recombinant Human Beta-hexosaminidase subunit beta (HEXB)

  • 中文名稱：
    人HEXB重組蛋白
  • 貨號(hào)：
    CSB-YP010316HU
  • 規(guī)格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人HEXB重組蛋白
  • 貨號(hào)：
    CSB-EP010316HU-B
  • 規(guī)格：
  • 來源：
    E.coli
  • 共軛：
    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：
  • 中文名稱：
    人HEXB重組蛋白
  • 貨號(hào)：
    CSB-BP010316HU
  • 規(guī)格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人HEXB重組蛋白
  • 貨號(hào)：
    CSB-MP010316HU
  • 規(guī)格：
  • 來源：
    Mammalian cell
  • 其他：

產(chǎn)品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 別名：
    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
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length of Mature Protein
  • 表達(dá)區(qū)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
  • 蛋白標(biāo)簽：
    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.
  • 產(chǎn)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.
  • 復(fù)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.
  • 儲(chǔ)存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質(zhì)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.
  • 貨期：
    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.
  • 注意事項(xiàng)：
    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.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)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 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.
  • 基因功能參考文獻(xiàn)：
    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

    顯示更多

    收起更多

  • 相關(guān)疾病：
    GM2-gangliosidosis 2 (GM2G2)
  • 亞細(xì)胞定位：
    Lysosome. Cytoplasmic vesicle, secretory vesicle, Cortical granule.
  • 蛋白家族：
    Glycosyl hydrolase 20 family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 4879

    OMIM: 268800

    KEGG: hsa:3074

    STRING: 9606.ENSP00000261416

    UniGene: Hs.69293



主站蜘蛛池模板： 国产成人综合久久免费| 国产精品久久无码不卡黑寡妇| 日本欧美大码aⅴ在线播放| 首页 国产 亚洲 丝袜图片区| 国产精品门事件av| 少妇性l交大片欧洲热妇乱xxx| 亚洲视频在线观看| 久久久无码精品亚洲日韩啪啪网站 | 亚洲自偷自拍另类小说| 亚洲成a人片在线观看无码专区| 麻豆国产97在线 | 欧美| 精品毛片乱码1区2区3区| 99久在线国内在线播放免费观看| 久久久久国产精品免费免费搜索| 亚洲最大无码中文字幕| 亚洲国产成人爱av在线播放| 日韩亚洲国产中文永久| 国产精品毛片久久久久久久| 日日碰狠狠躁久久躁96avv| 成人片无码免费播放| 乱人伦中文无码视频| 在线观看特色大片免费网站| 国内精品久久久久影院嫩草| 亚洲人成手机电影网站| 欧美午夜精品久久久久免费视| 女人高潮流白浆视频| 草草久久97超级碰碰碰| 成人污污污www网站免费| 国产精品久久久久久人妻精品| 久久伊人五月丁香狠狠色| 狼群社区www中文视频| 精品国产在天天在线观看| 日韩一区二区a片免费观看| 国产未发育呦交视频| 国产无遮挡又黄又爽动态图| 老熟女重囗味hdxx70星空| 国产男女无遮挡猛进猛出| 92午夜少妇极品福利无码电影| 久久丫精品系列| 人妻少妇精品无码专区二区| 久久久www成人免费看片|