製品の概要

  • 製品名
  • 製品の詳細
    Mouse 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
  • 免疫原

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

  • ポジティブ・コントロール
    • Lysate from 293T cells transfected with ARSA

製品の特性

アプリケーション

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.

アプリケーション Abreviews 特記事項
WB
  • 追加情報
    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.
  • 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

    画像

    • 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?)

    プロトコール

    参考文献

    ab67089 has not yet been referenced specifically in any publications.

    レビューと Q&A

    There are currently no Customer reviews or Questions for ab67089.
    Please use the links above to contact us or submit feedback about this product.

    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

    登録