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  4. ALG9 Antibody, FITC conjugated

ALG9 Antibody, FITC conjugated

  • 中文名稱：
    ALG9兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA867161LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) ALG9 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    ALG9
  • 別名：
    ALG9 antibody; ALG9_HUMAN antibody; Alpha-1,2-mannosyltransferase ALG9 antibody; Asparagine-linked glycosylation protein 9 homolog antibody; Disrupted in bipolar disorder protein 1 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Alpha-1,2-mannosyltransferase ALG9 protein (434-618AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 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
  • 產品提供形式：
    Liquid
  • 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：
    Catalyzes the transfer of mannose from Dol-P-Man to lipid-linked oligosaccharides.
  • 基因功能參考文獻：
    1. ALG9 is upregulated in peripheral blood mononuclear cells of galactosaemia patients. PMID: 26733289
    2. Our study shows that some pathogenic variants in ALG9 can present as a lethal skeletal dysplasia with visceral malformations as the most severe phenotype PMID: 25966638
    3. Due to the ALG9 deficiency, cells accumulated the lipid-linked oligosaccharides Man(6)GlcNAc(2)-PP-dolichol and Man(8)GlcNAc(2)-PP-dolichol. PMID: 19451548
  • 相關疾病：
    Congenital disorder of glycosylation 1L (CDG1L); Gillessen-Kaesbach-Nishimura syndrome (GIKANIS)
  • 亞細胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Glycosyltransferase 22 family
  • 組織特異性：
    Ubiquitously expressed; with highest levels in heart, liver and pancreas.
  • 數據庫鏈接：

    HGNC: 15672

    OMIM: 263210

    KEGG: hsa:79796

    STRING: 9606.ENSP00000435517

    UniGene: Hs.745155



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