Overview

  • Product nameRecombinant Human ARSB protein
  • Protein lengthProtein fragment

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP15848
    • SpeciesHuman
    • SequenceFGYLLGSEDYYSHERCTLIDALNVTRCALDFRDGEEVATGYKNMYSTNIF TKRAIALITNHPPEKPLFLYLALQSVHEPLQVPEEYLKPYDFIQDKNRHH
    • Molecular weight37 kDa including tags
    • Amino acids166 to 265

Specifications

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.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names
    • Arsb
    • ARSB_HUMAN
    • Arylsulfatase B
    • ArylsulfataseB
    • ASB
    • G4S
    • MPS6
    • N acetylgalactosamine 4 sulfatase
    • N-acetylgalactosamine-4-sulfatase
    see all
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the sulfatase family.
  • Post-translational
    modifications
    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).
  • Cellular localizationLysosome.
  • Information by UniProt

Recombinant Human ARSB protein images

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

References for Recombinant Human ARSB protein (ab117054)

ab117054 has not yet been referenced specifically in any publications.

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