Overview

  • Product nameAnti-ARSB antibody - C-terminalSee all ARSB primary antibodies ...
  • Description
    Rabbit polyclonal to ARSB - C-terminal
  • Tested applicationsWB, ELISA more details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    KLH conjugated synthetic peptide selected from the C-terminal region of human ARSB.

  • Positive control
    • HepG2 cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab82416 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Notes
WB 1/50 - 1/100. Predicted molecular weight: 60 kDa.
ELISA 1/1000.

Target

  • 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.
  • Target information above from: UniProt accession P15848 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • Database links
  • Alternative names
    • 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 - C-terminal images

  • Anti-ARSB antibody - C-terminal (ab82416) at 1/50 dilution + HepG2 lysate at 35 µg

    Predicted band size : 60 kDa
    Observed band size : 65 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 50 kDa. We are unsure as to the identity of these extra bands.

References for Anti-ARSB antibody - C-terminal (ab82416)

ab82416 has not yet been referenced specifically in any publications.

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