Recombinant人ARSB protein (ab117054)

概述

  • 产品名称Recombinant人ARSB protein
  • 蛋白长度Protein fragment

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP15848
    • 种属Human
    • 序列FGYLLGSEDYYSHERCTLIDALNVTRCALDFRDGEEVATGYKNMYSTNIF TKRAIALITNHPPEKPLFLYLALQSVHEPLQVPEEYLKPYDFIQDKNRHH
    • 分子量37 kDa including tags
    • 氨基酸166 to 265

技术指标

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.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • Arsb
    • ARSB_HUMAN
    • Arylsulfatase B
    • ArylsulfataseB
    • ASB
    • G4S
    • MPS6
    • N acetylgalactosamine 4 sulfatase
    • N-acetylgalactosamine-4-sulfatase
    see all
  • 疾病相关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.
  • 序列相似性Belongs to the sulfatase family.
  • 翻译后修饰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).
  • 细胞定位Lysosome.
  • Information by UniProt

Recombinant Human ARSB protein 图像

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

Recombinant Human ARSB protein (ab117054)参考文献

ab117054 has not yet been referenced specifically in any publications.

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