概述

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

    Full length protein, corresponding to amino acids 1-507 of Human ARSA

  • 阳性对照
    • Lysate from 293T cells transfected with ARSA

性能

应用

Our Abpromise guarantee covers the use of ab67089 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
  • 应用说明WB: Use at an assay dependent dilution. Detects a band of approximately 70 kDa (predicted molecular weight: 54 kDa).
    Detection Pair.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 功能Hydrolyzes cerebroside sulfate.
    • 疾病相关Defects in ARSA are a cause of leukodystrophy metachromatic (MLD) [MIM:250100]. MLD is a disease due to a lysosomal storage defect. It is characterized by intralysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues, with a diffuse loss of myelin in the central nervous system. Progressive demyelination causes a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Three forms of the disease can be distinguished according to the age at onset: late-infantile, juvenile and adult.
      Arylsulfatase A 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
      • arsA antibody
      • ARSA_HUMAN antibody
      • arylsulfatase A antibody
      • Arylsulfatase A component C antibody
      • As 2 antibody
      • AS A antibody
      • As2 antibody
      • ASA antibody
      • AW212749 antibody
      • C230037L18Rik antibody
      • Cerebroside-sulfatase antibody
      • metachromatic leucodystrophy antibody
      • MGC125207 antibody
      • MLD antibody
      • OTTHUMP00000196546 antibody
      • OTTHUMP00000196548 antibody
      • TISP73 antibody
      see all

    Anti-ARSA antibody 图像

    • All lanes : Anti-ARSA antibody (ab67089)

      Lane 1 : ARSA transfected 293T cells lysate
      Lane 2 : non transfected 293T cells lysate

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

      Predicted band size : 54 kDa
      Observed band size : 70 kDa (why is the actual band size different from the predicted?)

    Anti-ARSA antibody (ab67089)参考文献

    ab67089 has not yet been referenced specifically in any publications.

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