Anti-ARSB抗体- C-terminal (ab82416)

概述

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

    KLH conjugated synthetic peptide selected from the C-terminal region of human ARSB.

  • 阳性对照
    • HepG2 cell lysate.

性能

应用

Our Abpromise guarantee covers the use of ab82416 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 1/50 - 1/100. Predicted molecular weight: 60 kDa.
ELISA 1/1000.

靶标

  • 疾病相关Defects in ARSB are the cause of mucopolysaccharidosis type 6 (MPS6) [MIM:253200]; also known as Maroteaux-Lamy syndrome. MPS6 is an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of dermatan sulfate. Clinical features can include abnormal growth, short stature, stiff joints, skeletal malformations, corneal clouding, hepatosplenomegaly, and cardiac abnormalities. A wide variation in clinical severity is observed.
    Arylsulfatase B activity is defective in multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a disorder characterized by decreased activity of all known sulfatases. MSD is due to defects in SUMF1 resulting in the lack of post-translational modification of a highly conserved cysteine into 3-oxoalanine. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.
  • 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. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD).
  • 细胞定位Lysosome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Arsb antibody
    • ARSB_HUMAN antibody
    • Arylsulfatase B antibody
    • ArylsulfataseB antibody
    • ASB antibody
    • G4S antibody
    • MPS6 antibody
    • N acetylgalactosamine 4 sulfatase antibody
    • N-acetylgalactosamine-4-sulfatase antibody
    see all

Anti-ARSB antibody - C-terminal 图像

  • Anti-ARSB antibody - C-terminal (ab82416) at 1/50 dilution + HepG2 lysate at 35 µg

    Predicted band size : 60 kDa
    Observed band size : 65 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 50 kDa. We are unsure as to the identity of these extra bands.

Anti-ARSB antibody - C-terminal (ab82416)参考文献

ab82416 has not yet been referenced specifically in any publications.

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