概述

  • 产品名称Anti-IDUA抗体
    参阅全部 IDUA 一抗
  • 描述
    兔多克隆抗体to IDUA
  • 经测试应用适用于: WB, ICC/IFmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide conjugated to KLH, corresponding to a region within internal sequence amino acids 244-274 of Human IDUA (NP_000194.2).

  • 阳性对照
    • HeLa cell lysate. IF/ICC: HepG2 cell line.

性能

应用

Our Abpromise guarantee covers the use of ab103949 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 1/100 - 1/500. Predicted molecular weight: 73 kDa.
ICC/IF Use a concentration of 10 µg/ml.

靶标

  • 相关性IDUA is an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Defects in IDUA are the cause of mucopolysaccharidosis type 1H (MPS1H) also known as Hurler syndrome, mucopolysaccharidosis type 1H/S (MPS1H/S) also known as Hurler-Scheie syndrome and mucopolysaccharidosis type 1S (MPS1S) also known as Scheie syndrome. MPS1S is a mild form whilst MPS1H is a severe form of this rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate.
  • 细胞定位Lysosome
  • 数据库链接
  • 别名
    • Alpha L iduronidase antibody
    • IDA antibody
    • Iduronidase alpha L antibody
    • MPS1 antibody

Anti-IDUA antibody 图像

  • ab103949 stained HepG2 cells. The cells were 4% formaldehyde fixed for 10 minutes at room temperature and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1hour at room temperature to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab103949 at 10µg/ml) overnight at +4°C. The secondary antibody (pseudo-colored green) was Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) preadsorbed (ab150081) used at a 1/1000 dilution for 1hour at room temperature. Alexa Fluor® 594 WGA was used to label plasma membranes (pseudo-colored red) at a 1/200 dilution for 1hour at room temperature. DAPI was used to stain the cell nuclei (pseudo-colored blue) at a concentration of 1.43µM for 1hour at room temperature.

  • Anti-IDUA antibody (ab103949) at 1/100 dilution + HeLa cell lysate at 35 µg

    Predicted band size : 73 kDa

Anti-IDUA antibody (ab103949)参考文献

ab103949 has not yet been referenced specifically in any publications.

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