Human ARSB ELISA Kit (ab277466)
Key features and details
- Sensitivity: 0.32 ng/ml
- Range: 0.307 ng/ml - 75 ng/ml
- Sample type: Cell culture supernatant, Plasma, Serum
- Detection method: Colorimetric
- Assay type: Sandwich (quantitative)
- Reacts with: Human
Overview
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Product name
Human ARSB ELISA Kit -
Detection method
Colorimetric -
Precision
Intra-assay Sample n Mean SD CV% Overall Inter-assay Sample n Mean SD CV% Overall -
Sample type
Cell culture supernatant, Serum, Plasma -
Assay type
Sandwich (quantitative) -
Sensitivity
0.32 ng/ml -
Range
0.307 ng/ml - 75 ng/ml -
Recovery
Sample specific recovery Sample type Average % Range Serum 140 128% - 148% Plasma 141.5 133% - 146% Cell culture media 144 136% - 147% -
Assay duration
Multiple steps standard assay -
Species reactivity
Reacts with: Human -
Product overview
Human ARSB ELISA Kit (ab277466) is an in-vitro enzyme-linked immunosorbent assay for the quantitative measurement of Human ARSB in serum, plasma and cell culture supernatants.
This assay employs an antibody specific for Human ARSB coated on a 96-well plate. Standards and samples are pipetted into the wells and Human ARSB present in a sample is bound to the wells by the immobilized antibody. The wells are washed, and biotinylated anti-Human ARSB antibody is added. After washing away unbound biotinylated antibody, HRP-conjugated streptavidin is pipetted to the wells. The wells are again washed, a TMB substrate solution is added to the wells and color develops in proportion to the amount of Human ARSB bound. The Stop Solution changes the color from blue to yellow, and the intensity of the color is measured at 450 nm.
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Tested applications
Suitable for: Sandwich ELISAmore details -
Platform
Pre-coated microplate (12 x 8 well strips)
Properties
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Storage instructions
Store at -20°C. Please refer to protocols. -
Components 1 x 96 tests 20X Wash Buffer Concentrate 1 x 25ml 250X HRP-Streptavidin Concentrate 1 x 200µl 5X Assay Diluent 1 x 15ml Biotinylated Anti-Human ARSB Antibody 2 vials Anti-Human ARSB coated Microplate 1 unit Human ARSB standard protein (Lyophilized) 2 vials Stop Solution 1 x 8ml TMB One-Step Substrate Reagent 1 x 12ml -
Research areas
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Involvement in disease
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. -
Sequence similarities
Belongs 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 localization
Lysosome. - Information by UniProt
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Alternative names
- Arsb
- ARSB_HUMAN
- Arylsulfatase B
see all -
Database links
- Entrez Gene: 411 Human
- Omim: 611542 Human
- SwissProt: P15848 Human
- Unigene: 149103 Human
- Unigene: 604199 Human
Images
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Example data
These standard curves are for demonstration only. A standard curve must be run with each assay.