Human ARSB ELISA Kit | ARSB elisa kit

Human ARSB PicoKine ELISA Kit

Cat. Num. AAA1752683

• Gene Names: ARSB; ASB; G4S; MPS6
• Reactivity: Human
• Synonyms: ARSB; Human ARSB PicoKine ELISA Kit; Human ARSB; Arylsulfatase B; ASB; N-acetylgalactosamine-4-sulfatase; G4S; ARSB elisa kit

• Reactivity: Human
• Specificity: Natural and recombinant Human ARSB
• Sequence Length: 533

• Samples: Cell culture supernates, serum and plasma (heparin, EDTA, citrate)
• Detection Range: 15.6pg/ml-1000pg/ml
• Sensitivity: <10pg/ml
• Intra-assay Precision: Intra-Assay Precision (Precision within an assay): Three samples of known concentration were tested on one plate to assess intra-assay precision.
• Inter-assay Precision: Inter-Assay Precision (Precision across assays):Three samples of known concentration were tested in separate assays to assess inter-assay precision.
• Preparation and Storage: Store at 4 degree C for 6 months. Store at -20 degree C for 12 months. Avoid multiple freeze-thaw cycles.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for ARSB elisa kit

Principle of the Assay: The Picokine™ Human ARSB Pre-Coated ELISA (Enzyme-Linked Immunosorbent Assay) kit is a solid phase immunoassay specially designed to measure Human ARSB with a 96-well strip plate that is pre-coated with antibody specific for ARSB. The detection antibody is a biotinylated antibody specific for ARSB. The capture antibody is a monoclonal antibody from mouse, the detection antibody is a biotinylated polyclonal antibody from goat. The kit contains recombinant Human ARSB with immunogen: Expression system for standard: NSO, Immunogen sequence: S37-M533. The kit is analytically validated with ready to use reagents. To measure Human ARSB, add standards and samples to the wells, then add the biotinylated detection antibody. Wash the wells with PBS or TBS buffer, and add Avidin-Biotin-Peroxidase Complex (ABC-HRP). Wash away the unbounded ABC-HRP with PBS or TBS buffer and add TMB. TMB is substrate to HRP and will be catalyzed to produce a blue color product, which changes into yellow after adding acidic stop solution. The density of the yellow product is linearly proportional to Human ARSB in the sample. Read the density of the yellow product in each well using a plate reader, and benchmark the sample wells' readings against the standard curve to determine the concentration of Human ARSB in the sample.!!Background/Introduction: Arylsulfatase B is an enzyme associated with mucopolysaccharidosis VI. It is mapped to 5q14.1. Arylsulfatase B encoded by this gene belongs to the sulfatase family. The arylsulfatase B homodimer hydrolyzes sulfate groups of N-Acetyl-D-galactosamine, chondriotin sulfate, and dermatan sulfate. The protein is targeted to the lysozyme. Mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder resulting from a deficiency of arylsulfatase B. Two alternatively spliced transcript variants encoding distinct isoforms have been found for this gene.

NCBI and Uniprot Product Information

• NCBI GI #: 38569405
• NCBI GeneID: 411
• NCBI Accession #: NP_000037
• NCBI GenBank Nucleotide #: NP_000037.2
• UniProt Accession #: P15848
• NCBI Official Full Name: arylsulfatase B isoform 1
• NCBI Official Synonym Full Names: arylsulfatase B
• NCBI Official Symbol: ARSB
• NCBI Official Synonym Symbols: ASB; G4S; MPS6
• NCBI Protein Information: arylsulfatase B
• UniProt Protein Name: Arylsulfatase B
• Protein Family: Arylsulfatase
• UniProt Gene Name: ARSB
• UniProt Synonym Gene Names: ASB; G4S
• UniProt Entry Name: ARSB_HUMAN

NCBI Description

Arylsulfatase B encoded by this gene belongs to the sulfatase family. The arylsulfatase B homodimer hydrolyzes sulfate groups of N-Acetyl-D-galactosamine, chondriotin sulfate, and dermatan sulfate. The protein is targeted to the lysozyme. Mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder resulting from a deficiency of arylsulfatase B. Two alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, Dec 2016]

Uniprot Description

ARSB: Defects in ARSB are the cause of mucopolysaccharidosis type 6 (MPS6); 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). A clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Arylsulfatase B activity is impaired in multiple sulfatase deficiency due to mutations in SUMF1. SUMF1 mutations result in defective post-translational modification of ARSB at residue Cys- 91 that is not converted to 3-oxoalanine. Belongs to the sulfatase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.1.6.12; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation

Chromosomal Location of Human Ortholog: 5q14.1

Cellular Component: Golgi apparatus; lysosomal lumen; cell surface; rough endoplasmic reticulum; mitochondrion; endoplasmic reticulum lumen; lysosome

Molecular Function: arylsulfatase activity; N-acetylgalactosamine-4-sulfatase activity; metal ion binding

Biological Process: lysosomal transport; sphingolipid metabolic process; central nervous system development; glycosaminoglycan metabolic process; pathogenesis; post-translational protein modification; chondroitin sulfate metabolic process; cellular protein metabolic process; lysosome organization and biogenesis; response to estrogen stimulus; chondroitin sulfate catabolic process; response to methylmercury; carbohydrate metabolic process; autophagy; glycosphingolipid metabolic process; response to nutrient; response to pH

Disease: Mucopolysaccharidosis Type Vi