概述

  • 产品名称
    Anti-Galactosidase alpha抗体
    参阅全部 Galactosidase alpha 一抗
  • 描述
    兔多克隆抗体to Galactosidase alpha
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Non human primates
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human Galactosidase alpha.

    (Peptide available as ab91202.)

  • 阳性对照
    • This antibody gave a positive signal in Human Liver Tissue Lysate.

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 or -80°C. Avoid freeze / thaw cycle.
  • 存储溶液
    Preservative: 0.02% Sodium Azide
    Constituents: 1% BSA, PBS, pH 7.4
  • Concentration information loading...
  • 纯度
    Immunogen affinity purified
  • 克隆
    多克隆
  • 同种型
    IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab70520 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. Detects a band of approximately 49 kDa (predicted molecular weight: 49 kDa).

靶标

  • 疾病相关
    Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.
  • 序列相似性
    Belongs to the glycosyl hydrolase 27 family.
  • 细胞定位
    Lysosome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AGAL_HUMAN antibody
    • Agalsidase alfa antibody
    • Alpha D galactosidase A antibody
    • Alpha D galactoside galactohydrolase 1 antibody
    • Alpha D galactoside galactohydrolase antibody
    • Alpha gal A antibody
    • Alpha galactosidase A antibody
    • Alpha-D-galactosidase A antibody
    • Alpha-D-galactoside galactohydrolase antibody
    • Alpha-galactosidase A antibody
    • GALA antibody
    • Galactosidase, alpha antibody
    • GLA antibody
    • GLA protein antibody
    • Melibiase antibody
    see all

图片

  • Anti-Galactosidase alpha antibody (ab70520) at 1 µg/ml + Human liver tissue lysate - total protein (ab29889) at 10 µg

    Secondary
    Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 49 kDa
    Observed band size : 49 kDa
    Additional bands at : 47 kDa (possible cleavage fragment).

    Galactosidase alpha contains a 31 amino acid signal peptide. We hypothesize that the 47 kDa band represents the mature protein. Some batches of this product also recognise a band at 100 kDa in Western Blot, we are unsure of the identity of this band.

实验方案

文献

This product has been referenced in:
  • Citro V  et al. Identification of an Allosteric Binding Site on Human Lysosomal Alpha-Galactosidase Opens the Way to New Pharmacological Chaperones for Fabry Disease. PLoS One 11:e0165463 (2016). WB . Read more (PubMed: 27788225) »
  • Sienkiewicz AE  et al. Aberrant glycosylation in the human trabecular meshwork. Proteomics Clin Appl 8:130-42 (2014). ELISA . Read more (PubMed: 24458570) »

See all 3 Publications for this product

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