概述

  • 产品名称Anti-ARSA抗体
    参阅全部 ARSA 一抗
  • 描述
    兔多克隆抗体to ARSA
  • 经测试应用适用于: IHC-P, WB, ELISAmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat
  • 免疫原

    Synthetic peptide derived from an internal sequence of human ARSA.

  • 阳性对照
    • Human cervix carcinoma tissue. Jurkat and COLO 205 cell extracts.

性能

应用

Our Abpromise guarantee covers the use of ab74804 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
IHC-P 1/50 - 1/100.
WB 1/500 - 1/1000. Predicted molecular weight: 54 kDa.
ELISA 1/20000.

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

  • ab74804 at 1/50 dilution staining ARSA in human cervix carcinoma by Immunohistochemistry, Paraffin-embedded tissue, in the absence or presence of the immunising peptide.
  • All lanes : Anti-ARSA antibody (ab74804) at 1/500 dilution

    Lane 1 : Jurkat cell extracts
    Lane 2 : COLO 205 cell extracts
    Lane 3 : Jurkat cell extracts with immunising peptide at 10 µg

    Lysates/proteins at 30 µg per lane.


    Predicted band size : 54 kDa
    Observed band size : 54 kDa
    Additional bands at : 30 kDa. We are unsure as to the identity of these extra bands.

Anti-ARSA antibody (ab74804)参考文献

ab74804 has not yet been referenced specifically in any publications.

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