製品の概要

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

    Synthetic peptide corresponding to a region within internal sequence amino acids 300-349 (TDNGGQTRSG GNNWPLRGRK GTLWEGGIRG TGFVASPLLK QKGVKSRELM) of Mouse ARSB (NP_033842).

  • ポジティブ・コントロール
    • Mouse kidney lysate.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab115894 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. Predicted molecular weight: 60 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

ターゲット情報

  • 関連疾患Defects in ARSB are the cause of mucopolysaccharidosis type 6 (MPS6) [MIM:253200]; also known as Maroteaux-Lamy syndrome. MPS6 is an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of dermatan sulfate. Clinical features can include abnormal growth, short stature, stiff joints, skeletal malformations, corneal clouding, hepatosplenomegaly, and cardiac abnormalities. A wide variation in clinical severity is observed.
    Arylsulfatase B 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
    • Arsb antibody
    • ARSB_HUMAN antibody
    • Arylsulfatase B antibody
    • ArylsulfataseB antibody
    • ASB antibody
    • G4S antibody
    • MPS6 antibody
    • N acetylgalactosamine 4 sulfatase antibody
    • N-acetylgalactosamine-4-sulfatase antibody
    see all

Anti-ARSB antibody 画像

  • Anti-ARSB antibody (ab115894) at 1 µg/ml + Mouse kidney lysate at 10 µg

    Predicted band size : 60 kDa

Anti-ARSB antibody (ab115894) 使用論文

ab115894 has not yet been referenced specifically in any publications.

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