概述

  • 产品名称Anti-SGSH抗体
    参阅全部 SGSH 一抗
  • 描述
    兔多克隆抗体to SGSH
  • 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, Dog
  • 免疫原

    Recombinant fragment, corresponding a region within amino acids 44-200 of Human SGSH

  • 阳性对照
    • 293T whole cell lysate

性能

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应用

Our Abpromise guarantee covers the use of ab96030 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 1/500 - 1/3000. Predicted molecular weight: 57 kDa.

靶标

  • 疾病相关Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival.
  • 序列相似性Belongs to the sulfatase family.
  • 翻译后修饰The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
  • 细胞定位Lysosome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Heparan sulfate sulfatase antibody
    • Heparan sulphate sulphatase antibody
    • HSS antibody
    • MPS 3A antibody
    • MPS3 A antibody
    • MPS3A antibody
    • Mucopolysaccharidosis type IIIA antibody
    • N sulfoglucosamine sulfohydrolase (sulfamidase) antibody
    • N sulfoglucosamine sulfohydrolase antibody
    • N-sulphoglucosamine sulphohydrolase antibody
    • SFMD antibody
    • SGSH antibody
    • SPHM_HUMAN antibody
    • Sulfoglucosamine sulfamidase antibody
    • Sulphamidase antibody
    • Sulphoglucosamine sulphamidase antibody
    see all

Anti-SGSH antibody 图像

  • Anti-SGSH antibody (ab96030) at 1/1000 dilution + 293T whole cell lysate at 30 µg

    Predicted band size : 57 kDa

Anti-SGSH antibody (ab96030)参考文献

ab96030 has not yet been referenced specifically in any publications.

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