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ALG8 Antibody

  • 中文名稱:
    ALG8兔多克隆抗體
  • 貨號:
    CSB-PA725690
  • 規格:
    ¥1100
  • 圖片:
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA725690(ALG8 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using CSB-PA725690(ALG8 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
  • 別名:
    ALG8 antibody; HUSSY-02Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase antibody; EC 2.4.1.265 antibody; Asparagine-linked glycosylation protein 8 homolog antibody; Dol-P-Glc:Glc(1)Man(9)GlcNAc(2)-PP-dolichyl alpha-1,3-glucosyltransferase antibody; Dolichyl-P-Glc:Glc1Man9GlcNAc2-PP-dolichyl glucosyltransferase antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse
  • 免疫原:
    Fusion protein of Human ALG8
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen affinity purification
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,IHC
  • 推薦稀釋比:
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    IHC 1:25-1:100
  • 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 second 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 Glc(1)Man(9)GlcNAc(2)-PP-Dol before it is transferred to the nascent peptide. Required for PKD1/Polycystin-1 maturation and localization to the plasma membrane of the primary cilia.
  • 基因功能參考文獻:
    1. used whole exome sequencing in a discovery cohort of 102 unrelated patients who were excluded for mutations in the 2 most common polycystic liver disease genes, PRKCSH and SEC63, to identify heterozygous loss-of-function mutations in 3 additional genes, ALG8, GANAB, and SEC61B. Similarly to PRKCSH and SEC63, these genes encode proteins that are integral to the protein biogenesis pathway in the endoplasmic reticulum. PMID: 28375157
    2. In ALG8-CDG, isoelectric focusing of transferrin in serum or plasma shows an abnormal sialotransferrin pattern. The diagnosis is confirmed by mutation analysis in ALG8; all patients reported so far had point mutations or small deletions PMID: 26066342
    3. We reviewed the clinical features in all nine previously reported patients diagnosed with ALG8-disorder of glycosylation with a special focus on their skin signs. PMID: 24555185
    4. Severe ALG8 congenital disorder of glycosylation(CDG-Ih) is associated with homozygosity for two novel missense mutations in exon 8 of ALG8 PMID: 22306853
    5. ALG8 mutations expand the clinical spectrum of congenital disorder of glycosylation type Ih. PMID: 19648040
    6. ALG8 splice site mutations and missense mutations causing ALG8 deficiency in patients with congenital disorders of glycosylation type Ih. PMID: 15235028

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  • 相關疾病:
    Congenital disorder of glycosylation 1H (CDG1H)
  • 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: 23161

    OMIM: 608103

    KEGG: hsa:79053

    STRING: 9606.ENSP00000299626

    UniGene: Hs.503368



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