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ALG6 Antibody

  • 货号:
    CSB-PA782102
  • 规格:
    ¥1100
  • 图片:
    • Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane: Human lymphoma tissue, Primary antibody: CSB-PA782102(ALG6 Antibody) at dilution 1/350, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 30 seconds
  • 其他:

产品详情

  • Uniprot No.:
    Q9Y672
  • 基因名:
    ALG6
  • 别名:
    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
  • 反应种属:
    Human,Mouse,Rat
  • 免疫原:
    Synthetic peptide of Human ALG6
  • 免疫原种属:
    Homo sapiens (Human)
  • 标记方式:
    Non-conjugated
  • 抗体亚型:
    IgG
  • 纯化方式:
    Antigen affinity purification
  • 浓度:
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液:
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式:
    Liquid
  • 应用范围:
    ELISA,WB
  • 推荐稀释比:
    Application Recommended Dilution
    ELISA 1:1000-1:2000
    WB 1:200-1:1000
  • Protocols:
  • 储存条件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期:
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献:
    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
  • 相关疾病:
    Congenital disorder of glycosylation 1C (CDG1C)
  • 亚细胞定位:
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族:
    ALG6/ALG8 glucosyltransferase family
  • 数据库链接:

    HGNC: 23157

    OMIM: 603147

    KEGG: hsa:29929

    STRING: 9606.ENSP00000360149

    UniGene: Hs.258501