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. Home
  2. 抗體
  3. 多克隆抗體
  4. HEXB Antibody

HEXB Antibody

  • 中文名稱：
    HEXB兔多克隆抗體
  • 貨號(hào)：
    CSB-PA010316LA01HU
  • 規(guī)格：
    ￥440
  • 圖片：
    • IHC image of CSB-PA010316LA01HU diluted at 1:300 and staining in paraffin-embedded human colon cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) HEXB Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    Beta hexosaminidase beta chain antibody; Beta hexosaminidase subunit beta antibody; Beta N acetylhexosaminidase antibody; Beta-hexosaminidase subunit beta chain A antibody; Beta-N-acetylhexosaminidase subunit beta antibody; Cervical cancer proto oncogene 7 protein antibody; Cervical cancer proto-oncogene 7 protein antibody; ENC 1AS antibody; Epididymis luminal protein 248 antibody; HCC 7 antibody; HCC-7 antibody; HCC7 antibody; HEL 248 antibody; HEX B antibody; Hexb antibody; HEXB_HUMAN antibody; Hexosaminidase B (beta polypeptide) antibody; Hexosaminidase B antibody; Hexosaminidase subunit B antibody; HexosaminidaseB antibody; N acetyl beta glucosaminidase antibody; N-acetyl-beta-glucosaminidase subunit beta antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Beta-hexosaminidase subunit beta protein (408-528AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated

    本頁面中的產(chǎn)品，HEXB Antibody (CSB-PA010316LA01HU)，的標(biāo)記方式是Non-conjugated。對(duì)于HEXB Antibody，我們還提供其他標(biāo)記。見下表：

    可提供標(biāo)記
    標(biāo)記方式 貨號(hào) 產(chǎn)品名稱 應(yīng)用
    HRP CSB-PA010316LB01HU HEXB Antibody, HRP conjugated ELISA
    FITC CSB-PA010316LC01HU HEXB Antibody, FITC conjugated
    Biotin CSB-PA010316LD01HU HEXB Antibody, Biotin conjugated ELISA
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:200-1:500
  • Protocols：
  • 儲(chǔ)存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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： 无码中文字幕在线播放2| 日本强伦姧人妻一区二区| 在线成人精品国产区免费| 性欧美俄罗斯乱妇| 人与狗精品aa毛片| 国产午夜福利在线播放| 国产成人精品视频ⅴa片软件竹菊| 中文在线www天堂网| 99久久精品国产一区二区| 国产中老年妇女精品| 女厕厕露p撒尿八个少妇| 香港三日本三级少妇三级2021| 亚洲成在人线免费观看| 亚洲乱码日产精品bd在线观看| 日本无遮挡边做边爱边摸| 色午夜日本高清视频www| 亚洲国产精品久久久久爰| 国产香蕉尹人在线视频你懂的 | 婷婷久久综合九色综合88| 四虎www成人影院观看| 国产亚洲精品久久久久久入口| 亚洲图片自拍偷图区| 亚洲熟妇无码乱子av电影| 中文字幕乱码人妻综合二区三区| 性一交一乱一乱一视频| 欧洲美女熟乱av| 人人婷婷人人澡人人爽| 亚洲日韩精品射精日| 在线观看的网站| 18禁美女黄网站色大片免费看 | 亚洲国产欧美在线人成大黄瓜| 中文在线√天堂| 亚洲欧美另类久久久精品| 亚洲国产精品第一区二区 | 美女粉嫩饱满的一线天mp4| 精品高朝久久久久9999| 一本一道波多野结衣一区| 狠狠色噜噜狠狠狠777米奇小说| 欧美多人片高潮野外做片黑人 | 国产自在自线午夜精品| 久久99精品国产99久久6|