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B3GALT6 Antibody

  • 中文名稱：
    B3GALT6兔多克隆抗體
  • 貨號：
    CSB-PA002494GA01HU
  • 規(guī)格：
    ￥3,900
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
    B3GALT6
  • 別名：
    B3GALT6 antibody; Beta-1,3-galactosyltransferase 6 antibody; Beta-1,3-GalTase 6 antibody; Beta3Gal-T6 antibody; Beta3GalT6 antibody; EC 2.4.1.134 antibody; GAG GalTII antibody; Galactosyltransferase II antibody; Galactosylxylosylprotein 3-beta-galactosyltransferase antibody; UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 6 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human,Mouse,Rat
  • 免疫原：
    Human B3GALT6
  • 免疫原種屬：
    Homo sapiens (Human)
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA,WB
  • Protocols：
  • 儲存條件：
    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.

產品評價

靶點詳情

  • 功能：
    Beta-1,3-galactosyltransferase that transfers galactose from UDP-galactose to substrates with a terminal beta-linked galactose residue. Has a preference for galactose-beta-1,4-xylose that is found in the linker region of glycosaminoglycans, such as heparan sulfate and chondroitin sulfate. Has no activity towards substrates with terminal glucosamine or galactosamine residues.
  • 基因功能參考文獻：
    1. B3GALT6 encoding an enzyme involved in the biosynthesis of the GAG linker region is responsible for a severe skeletal dysplasia, spondyloepimetaphyseal dysplasia with joint laxity type 1. PMID: 23664117
    2. Genetic association between B3GALT6 and Ehlers-Danlos-syndrome-like connective tissue disorder in 3 families. PMID: 23664118
  • 相關疾?。?/div>
    Ehlers-Danlos syndrome, progeroid type, 2 (EDSP2); Spondyloepimetaphyseal dysplasia with joint laxity, 1, with or without fractures (SEMDJL1)
  • 亞細胞定位：
    Golgi apparatus, Golgi stack membrane; Single-pass type II membrane protein.
  • 蛋白家族：
    Glycosyltransferase 31 family
  • 組織特異性：
    Ubiquitous.
  • 數據庫鏈接：

    HGNC: 17978

    OMIM: 271640

    KEGG: hsa:126792

    STRING: 9606.ENSP00000368496

    UniGene: Hs.284284