製品の概要

  • 製品名
  • 製品の詳細
    Goat polyclonal to ARSA
  • アプリケーション
    適用あり: WB, ELISAmore details
  • 種交差性
    交差種: Mouse
    交差が予測される動物種: Rat, Human, Chimpanzee, Rhesus monkey
  • 免疫原

    Synthetic peptide:

    C-YDLSKDPGENYN

    , corresponding to internal sequence amino acids 429-440 of human ARSA (NP_000478.2)

  • ポジティブ・コントロール
    • Mouse testis lysates.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab77586 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 0.5 - 1.5 µg/ml. Detects a band of approximately 54 kDa (predicted molecular weight: 54 kDa).
ELISA Use at an assay dependent concentration.

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

  • Anti-ARSA antibody (ab77586) at 0.5 µg/ml + Mouse Testis lysate (in RIPA buffer) at 35 µg

    Predicted band size : 54 kDa
    Observed band size : 54 kDa

プロトコール

参考文献

ab77586 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 ab77586.
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