概述

  • 产品名称Anti-GCS1抗体
    参阅全部 GCS1 一抗
  • 描述
    兔多克隆抗体to GCS1
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Horse, Guinea pig, Cow, Cat, Dog, Zebrafish
  • 免疫原

    Synthetic peptide corresponding to a region within N terminal amino acids 144-193 (GPYGWEFHDG LSFGRQHIQD GALRLTTEFV KRPGGQHGGD WSWRVTVEPQ) of human GCS1 (NP_006293).

  • 阳性对照
    • HeLa cell lysate.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Preservative: None
    Constituents: 2% Sucrose, PBS
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 纯化说明ab82962 is purified by a peptide affinity chromatography method.
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

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应用

Our Abpromise guarantee covers the use of ab82962 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 92 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
ELISA Use at an assay dependent concentration.

ELISA titre using peptide based assay, 1:312500.

靶标

  • 功能Cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner.
  • 通路Glycan metabolism; N-glycan degradation.
  • 疾病相关Defects in MOGS are the cause of type IIb congenital disorder of glycosylation (CDGIIb) [MIM:606056]; also known as glucosidase I deficiency. CDGIIb is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms of the infant included hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course was progressive and the infant did not survive more than a few months.
  • 序列相似性Belongs to the glycosyl hydrolase 63 family.
  • 细胞定位Endoplasmic reticulum membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • EC 3.2.1.106 antibody
    • glucosidase I antibody
    • Mannosyl oligosaccharide glucosidase antibody
    • Mannosyl-oligosaccharide glucosidase antibody
    • Mogs antibody
    • MOGS_HUMAN antibody
    • Processing A glucosidase I antibody
    • Processing A-glucosidase I antibody
    see all

Anti-GCS1 antibody 图像

  • Anti-GCS1 antibody (ab82962) at 1 µg/ml + HeLa cell lysate at 10 µg

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 92 kDa
    Observed band size : 92 kDa
    Additional bands at : 24 kDa,50 kDa. We are unsure as to the identity of these extra bands.

Anti-GCS1 antibody (ab82962)参考文献

ab82962 has not yet been referenced specifically in any publications.

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