製品の概要

  • 製品名Anti-ARSA antibody
    ARSA 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to ARSA
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 400 - 500 of Human ARSA.

    (Peptide available as ab88153.)

  • ポジティブ・コントロール
    • This antibody gave a positive signal in the following lysates: Human Testis Tissue Lysate Jurkat whole cell lysate HepG2 Whole Cell Lysate

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab76890 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
WB Use a concentration of 1 µg/ml. Detects a band of approximately 54 kDa (predicted molecular weight: 54 kDa).

ターゲット情報

  • 機能Hydrolyzes cerebroside sulfate.
  • 関連疾患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.
  • 配列類似性Belongs to the sulfatase family.
  • 翻訳後修飾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).
  • 細胞内局在Lysosome.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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 画像

  • All lanes : Anti-ARSA antibody (ab76890) at 1 µg/ml

    Lane 1 : Human testis tissue lysate - total protein (ab30257)
    Lane 2 : Jurkat (Human T cell lymphoblast-like cell line) Whole Cell Lysate
    Lane 3 : HepG2 (Human hepatocellular liver carcinoma cell line) Whole Cell Lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 54 kDa
    Observed band size : 53 kDa (why is the actual band size different from the predicted?)


    Exposure time : 30 seconds

Anti-ARSA antibody (ab76890) 使用論文

ab76890 has not yet been referenced specifically in any publications.

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