製品の概要

  • 製品名Recombinant Human ARSB protein
  • タンパク質長Protein fragment

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号P15848
    • 生物種Human
    • 配列FGYLLGSEDYYSHERCTLIDALNVTRCALDFRDGEEVATGYKNMYSTNIF TKRAIALITNHPPEKPLFLYLALQSVHEPLQVPEEYLKPYDFIQDKNRHH
    • 分子量37 kDa including tags
    • 領域166 to 265

特性

Our Abpromise guarantee covers the use of ab117054 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

    Western blot

    SDS-PAGE

    ELISA

  • 製品の状態Liquid
  • 備考Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

関連情報

  • 別名
    • Arsb
    • ARSB_HUMAN
    • Arylsulfatase B
    • ArylsulfataseB
    • ASB
    • G4S
    • MPS6
    • N acetylgalactosamine 4 sulfatase
    • N-acetylgalactosamine-4-sulfatase
    see all
  • 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

Recombinant Human ARSB protein 画像

  • 12.5% SDS-PAGE showing ab117054 at approximately 36.63 kDa.
    Stained with Coomassie Blue.

Recombinant Human ARSB protein (ab117054) 使用論文

ab117054 has not yet been referenced specifically in any publications.

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