Anti-SGSH抗体(ab72617)

概述

  • 产品名称
  • 描述
    小鼠多克隆抗体to SGSH
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    SGSH (NP_000190.1, 1 a.a. ~ 502 a.a) full-length human protein.

  • 阳性对照
    • SGSH transfected 293T cell line

性能

应用

Our Abpromise guarantee covers the use of ab72617 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 - 1/1000. 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

图片

  • All lanes : Anti-SGSH antibody (ab72617) at 1/500 dilution

    Lane 1 : SGSH transfected 293T cell line
    Lane 2 : Non transfected 293T cell line

    Lysates/proteins at 25 µg per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution
    Developed using the ECL technique

    Predicted band size : 57 kDa
    Observed band size : 67 kDa (why is the actual band size different from the predicted?)

实验方案

文献

ab72617 has not yet been referenced specifically in any publications.

客户评价及客户问答

Thank you for contacting us. Unfortunately, the details of the transfection are considered proprietary and I am unable to provide a protocol for the preparation of the cell line. We may be able to add the transfected lysate to our catalog, so please le...

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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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