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  4. ALG6 Antibody

ALG6 Antibody

  • 中文名稱：
    ALG6兔多克隆抗體
  • 貨號：
    CSB-PA001603GA01HU
  • 規格：
    ￥3,900
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
    ALG6
  • 別名：
    ALG6 antibody; My046 antibody; Dolichyl pyrophosphate Man9GlcNAc2 alpha-1,3-glucosyltransferase antibody; EC 2.4.1.267 antibody; Asparagine-linked glycosylation protein 6 homolog antibody; Dol-P-Glc:Man(9)GlcNAc(2)-PP-Dol alpha-1,3-glucosyltransferase antibody; Dolichyl-P-Glc:Man9GlcNAc2-PP-dolichyl glucosyltransferase antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human,Mouse,Rat
  • 免疫原：
    Human ALG6
  • 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：
    Adds the first glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation. Transfers glucose from dolichyl phosphate glucose (Dol-P-Glc) onto the lipid-linked oligosaccharide Man(9)GlcNAc(2)-PP-Dol.
  • 基因功能參考文獻：
    1. ALG6-CDG has been now described in 89 patients. PMID: 27287710
    2. Five novel base substitutions in the hALG6 gene were also found: three in exon 5 (c.383T>C, c.390G>A, and c.429G>C) and two in a downstream intervening sequence (IVS5+17C/T and IVS5+34G/A). PMID: 21899441
    3. A frequent mild mutation in ALG6 may exacerbate the clinical severity of patients with congenital disorder of glycosylation Ia (CDG-Ia) caused by phosphomannomutase deficiency. PMID: 11875054
    4. Our findings extend the causes of CDG to larger DNA deletions and identify the first Japanese CDG-Ic mutation. PMID: 16321363
  • 相關疾病：
    Congenital disorder of glycosylation 1C (CDG1C)
  • 亞細胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族：
    ALG6/ALG8 glucosyltransferase family
  • 數據庫鏈接：

    HGNC: 23157

    OMIM: 603147

    KEGG: hsa:29929

    STRING: 9606.ENSP00000360149

    UniGene: Hs.258501



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