Anti-GCS1抗体(ab59956)

概述

  • 产品名称
  • 描述
    兔多克隆抗体to GCS1
  • 经测试应用
    适用于: ICC/IF, ELISA, WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide conjugated to KLH: 10-30 amino acids in length at the N terminal of the first 50 amino acids of human GCS1.

性能

  • 形式
    Liquid
  • 存放说明
    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term.
  • 存储溶液
    Preservative: 0.09% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • 纯度
    Protein G purified
  • 克隆
    多克隆
  • 同种型
    IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab59956 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
ICC/IF Use a concentration of 1 - 5 µg/ml.
ELISA 1/1000.
WB 1/100 - 1/500. Detects a band of approximately 92 kDa (predicted molecular weight: 92 kDa).

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

图片



  • Predicted band size : 92 kDa
    Observed band size : 92 kDa
  • ICC/IF image of ab59956 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab59956, 1µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

文献

ab59956 has not yet been referenced specifically in any publications.

客户评价及客户问答

Thank you for your reply. I will be helping you while Carolyn is out of the office.

How have you measured the exact MWs of the bands in the WB image? I only see an 80 and 140 kDa marker. And what gel percentage are you using?

The pr...

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Thank you for contacting Abcam regarding ab59956.


The immunogen is XXX with UniProt accession number Q13724. The database entry does not mention long or short form of the protein so I am not sure if it should recognize or not.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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