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B4GALT7 Antibody

  • 货号：
    CSB-PA890666LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Mouse brain tissue
      All lanes: B4GALT7 antibody at 3μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 38 kDa
      Observed band size: 38 kDa
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) B4GALT7 Polyclonal antibody
  • Uniprot No.：
    Q9UBV7
  • 基因名：
    B4GALT7
  • 别名：
    B4GALT7 antibody; XGALT1 antibody; UNQ748/PRO1478Beta-1,4-galactosyltransferase 7 antibody; Beta-1,4-GalTase 7 antibody; Beta4Gal-T7 antibody; b4Gal-T7 antibody; EC 2.4.1.- antibody; Proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I antibody; UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 7 antibody; UDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 7 antibody; UDP-galactose:beta-xylose beta-1,4-galactosyltransferase antibody; XGPT antibody; XGalT-1 antibody; Xylosylprotein 4-beta-galactosyltransferase antibody; EC 2.4.1.133 antibody; Xylosylprotein beta-1,4-galactosyltransferase antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Beta-1,4-galactosyltransferase 7 protein (52-327AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，B4GALT7 Antibody (CSB-PA890666LA01HU)，的标记方式是Non-conjugated。对于B4GALT7 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA890666LB01HU B4GALT7 Antibody, HRP conjugated ELISA
    FITC CSB-PA890666LC01HU B4GALT7 Antibody, FITC conjugated
    Biotin CSB-PA890666LD01HU B4GALT7 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:200-1:2000
  • 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：
    Required for the biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts.
  • 基因功能参考文献：
    1. The phenotypes described in this article caused by bi-allelic mutations in B4GALT7 would benefit from reclassification and loss of its current association with PEDS. These conditions would be better grouped with the other linkeropathies. PMID: 26940150
    2. Our findings demonstrate that B4GALT7 is the causative gene for LRS. The identification of a unique homozygous mutation argues in favor of a founder effect. B4GALT7 encodes a galactosyltransferase. PMID: 24755949
    3. identified two key structural features forming stacking interactions with the aglycone, and the hydrogen bond between the His(195) nitrogen backbone and the carbonyl group of the coumarinyl molecule to develop a tight binder of hbeta4GalT7 PMID: 25568325
    4. a Michaelis complex of a glycosyltransferase has been described, and it clearly suggests an SN2 type catalytic mechanism for the beta4GalT7 enzyme. PMID: 24052259
    5. Two evolutionary conserved motifs, (163)DVD(165) and (221)FWGWGREDDE(230), are central in the organization of the enzyme active site. PMID: 20843813
    6. Mutated ennzyme affects glycosaminoglycan synthesis and is involved Ehlers-Danlos syndrome. PMID: 20809901
    7. This study establishes the molecular basis for beta4GalT7 defects associated with altered GAG synthesis in Ehlers-Danlos syndrome. PMID: 20691685
    8. phosphorylation of Xyl on the C-2 position prevents GalT-I activity PMID: 15522873
    9. reduced beta4GalT-7 activity resulting in defective glycosylation of decorin and biglycan may be responsible for the complex molecular pathology in beta4GalT-7 deficient Ehlers-Danlos syndrome patients PMID: 16583246
    10. Study suggests an heparan sulfate-dependent basic mechanism behind the altered wound repair phenotype of beta4GalT-7-deficient Ehlers-Danlos syndrome patients. PMID: 18158310

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  • 相关疾病：
    Ehlers-Danlos syndrome with short stature and limb anomalies (EDSSLA)
  • 亚细胞定位：
    Golgi apparatus, Golgi stack membrane; Single-pass type II membrane protein. Note=Cis cisternae of Golgi stack.
  • 蛋白家族：
    Glycosyltransferase 7 family
  • 组织特异性：
    High expression in heart, pancreas and liver, medium in placenta and kidney, low in brain, skeletal muscle and lung.
  • 数据库链接：

    HGNC: 930

    OMIM: 130070

    KEGG: hsa:11285

    STRING: 9606.ENSP00000029410

    UniGene: Hs.455109