概述

  • 产品名称Anti-GNS抗体
    参阅全部 GNS 一抗
  • 描述
    小鼠多克隆抗体to GNS
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length human GNS (NP_002067.1).

  • 阳性对照
    • GNS transfected 293T lysate.

性能

应用

Our Abpromise guarantee covers the use of ab69971 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. Detects a band of approximately 75 kDa (predicted molecular weight: 63 kDa).

靶标

  • 疾病相关Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D) [MIM:252940]; also known as Sanfilippo D syndrome. MPS3D is a 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.
  • 序列相似性Belongs to the sulfatase family.
  • 翻译后修饰The form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species.
    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
    • 2610016K11Rik antibody
    • AU042285 antibody
    • C87209 antibody
    • G6S antibody
    • Glucosamine (N-acetyl) 6 sulfatase antibody
    • Glucosamine 6 sulfatase antibody
    • Glucosamine-6-sulfatase antibody
    • GNS antibody
    • GNS_HUMAN antibody
    • MGC21274 antibody
    • N acetylglucosamine 6 sulfatase [Precursor] antibody
    • N-acetylglucosamine-6-sulfatase antibody
    • N28088 antibody
    see all

Anti-GNS antibody 图像

  • All lanes : Anti-GNS antibody (ab69971) at 1/500 dilution

    Lane 1 : GNS transfected 293T lysate
    Lane 2 : Non transfected 293T lysate

    Lysates/proteins at 25 µg per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP at 1/2500 dilution

    Predicted band size : 63 kDa
    Observed band size : 75 kDa (why is the actual band size different from the predicted?)

Anti-GNS antibody (ab69971)参考文献

ab69971 has not yet been referenced specifically in any publications.

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