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PGM3 Monoclonal Antibody

  • 中文名稱：
    PGM3 Monoclonal Antibody
  • 貨號：
    CSB-MA998074
  • 規格：
    ￥1320
  • 圖片：
    • Western blotting analysis using phosphoglucomutase 3 antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with phosphoglucomutase 3 antibody and HRP-conjugated goat anti-mouse secondary antibody respectively.
  • 其他：

產品詳情

  • 產品名稱：
    Mouse anti-Homo sapiens (Human) PGM3 Monoclonal antibody
  • Uniprot No.：
  • 基因名：
    PGM3
  • 別名：
    PGM3; Phosphoglucomutase 3; AGM1; PAGM; Acetylglucosamine Phosphomutase; Phosphoacetylglucosamine Mutase; DKFZP434B187; EC 5.4.2.3; PGM 3; N-Acetylglucosamine-Phosphate Mutase 1; N-Acetylglucosamine-Phosphate Mutase; Phosphoglucomutase-3; EC 5.4.2; IMD23
  • 宿主：
    Mouse
  • 反應種屬：
    Human, Rat
  • 免疫原：
    Recombinant Human PGM3 protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Mouse IgG1
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    21C6
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS (pH 7.4) containing 50% glycerol, and 0.02% sodium azide.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, WB
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:2500
  • 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：
    Catalyzes the conversion of GlcNAc-6-P into GlcNAc-1-P during the synthesis of uridine diphosphate/UDP-GlcNAc, a sugar nucleotide critical to multiple glycosylation pathways including protein N- and O-glycosylation.
  • 基因功能參考文獻：
    1. Novel PGM3 Mutation Is Associated With a Severe Phenotype of Bone Marrow Failure, Severe Combined Immunodeficiency, Skeletal Dysplasia, and Congenital Malformations. PMID: 28543917
    2. study reports the first founder mutation in PGM3 gene (p.Glu340del) in twelve Tunisian PGM3 deficient patients belonging to three consanguineous families originating from a rural district in west central Tunisia PMID: 28704707
    3. PGM3 mutation identified in a patient with hyper IgE syndrome results in lack of glycosylation at Asn264 and altered glycosylation profile. PMID: 26687240
    4. Data indicate the effect of the phosphoglucomutase 3 (PGM3) mutation for four immunodeficient siblings in a Swedish family. PMID: 26482871
    5. define PGM3-CDG as a treatable immunodeficiency, document the power of whole-exome sequencing in gene discoveries for rare disorders, and illustrate the utility of genomic analyses in studying combined and variable phenotypes PMID: 24931394
    6. Impairment of PGM3 function leads to a novel primary (inborn) error of development and immunity because biallelic hypomorphic mutations are associated with impaired glycosylation and a hyper-IgE-like phenotype. PMID: 24698316
    7. Autosomal recessive hypomorphic PGM3 mutations underlie a disorder of severe atopy, immune deficiency, autoimmunity, intellectual disability, and hypomyelination. PMID: 24589341
    8. Polymorphic analysis of the human phosphoglucomutase-3 gene. PMID: 20221814
    9. PGM(3) is identical to AGM(1). PMID: 12174217

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  • 相關疾病：
    Immunodeficiency 23 (IMD23)
  • 蛋白家族：
    Phosphohexose mutase family
  • 組織特異性：
    Found in many tissues except lung. Relatively high expression in pancreas, heart, liver, and placenta, and relatively low expression in brain, skeletal muscle and kidney.
  • 數據庫鏈接：

    HGNC: 8907

    OMIM: 172100

    KEGG: hsa:5238

    STRING: 9606.ENSP00000425809

    UniGene: Hs.661665