Anti-ARSB antibody - C-terminal (ab82416)
- Product nameAnti-ARSB antibody - C-terminalSee all ARSB primary antibodies ...
- DescriptionRabbit polyclonal to ARSB - C-terminal
- Tested applicationsWB, ELISA more details
- Species reactivityReacts with: Human
KLH conjugated synthetic peptide selected from the C-terminal region of human ARSB.
- Positive controlHepG2 cell lysate.
- Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
- Storage bufferPreservative: 0.09% Sodium Azide
- Concentration information loading...
- PurityAmmonium Sulphate Precipitation
- Purification notesab82416 is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
- Clonality Polyclonal
- Research Areas
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.
|WB||WB: 1/50 - 1/100. Predicted molecular weight: 60 kDa.|
- 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.
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.
- Arsb antibodyARSB_HUMAN antibodyArylsulfatase B antibody
- ArylsulfataseB antibodyASB antibodyG4S antibodyMPS6 antibodyN acetylgalactosamine 4 sulfatase antibodyN-acetylgalactosamine-4-sulfatase antibody
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.