Anti-ARSB antibody - C-terminal (ab82416)

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 controlHepG2 cell lysate.

Properties

• FormLiquid
• Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
• Storage bufferPreservative: 0.09% Sodium Azide
  Constituents: PBS
• Concentration information loading...
• PurityAmmonium Sulphate Precipitation
• Purification notesab82416 is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
• Clonality Polyclonal
• IsotypeIgG
• Research Areas
  • Metabolism
  • Pathways and Processes
  • Metabolism processes
  • Autophagy and mitophagy

  • Cancer
  • Signal transduction
  • Autophagy

  • Tags & Cell Markers
  • Subcellular Markers
  • Organelles
  • Lysosome

Applications

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
  modificationsThe 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
  • Entrez Gene: 411 Human
  • Omim: 253200 Human
  • SwissProt: P15848 Human
  • Unigene: 149103 Human
  • Unigene: 604199 Human

• Alternative names
  Arsb antibodyARSB_HUMAN antibodyArylsulfatase B antibody

  ArylsulfataseB antibodyASB antibodyG4S antibodyMPS6 antibodyN acetylgalactosamine 4 sulfatase antibodyN-acetylgalactosamine-4-sulfatase antibody

  see all

Anti-ARSB antibody - C-terminal images

• 

  Western blot - ARSB antibody - C-terminal (ab82416)
  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.