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: WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant protein fragment corresponding to a region within amino acids 318-466 of Human SGSH

  • 阳性对照
    • Molt-4 whole cell lysate, OVCAR3 xenograft, A431 cells

性能

相关产品

应用

Our Abpromise guarantee covers the use of ab96029 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.
IHC-P 1/100 - 1/500.

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 (ab96029) at 1/1000 dilution + Molt-4 whole cell lysate at 30 µg

    Predicted band size : 57 kDa
  • Immunohistochemical analysis of formalin fixed paraffin embedded OVCAR3 xenograft, using ab96029 antibody at 1/100 dilution.

Anti-SGSH antibody (ab96029)参考文献

This product has been referenced in:
  • Duncan FJ  et al. Broad functional correction of molecular impairments by systemic delivery of scAAVrh74-hSGSH gene delivery in MPS IIIA mice. Mol Ther 23:638-47 (2015). ICC/IF . Read more (PubMed: 25592334) »
  • Boado RJ  et al. Insulin receptor antibody-sulfamidase fusion protein penetrates the primate blood-brain barrier and reduces glycosoaminoglycans in Sanfilippo type A cells. Mol Pharm 11:2928-34 (2014). WB . Read more (PubMed: 24949884) »

See all 2 Publications for this product

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