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Home/Reagents/Antibody/Primary antibody/Polyclonal Antibody/Rabbit pAb/ARSB Polyclonal Antibody
SKU: WP0884 Categories: ,

ARSB Polyclonal Antibody

Ucallm_WP0884-50

Price range: $268.00 through $328.00

SKU: WP0884-100
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Product details

Background:

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 targetted 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, Jul 2008],

Specifications

TargetARSB
ReactivityHuman, Mouse, Rat
ApplicationsWB, ELISA
MW(Calculated)
MW(Observed)58kD
Host SpeciesRabbit
IsotypeIgG
Conjugate/ModificationUnmodified
Modification
Recommended Dilution RatioWB 1:500-2000; ELISA 1:5000-20000
FormulationLiquid in PBS containing 50% glycerol,0.5% BSA and 0.02% sodium azide.
Source
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Purity
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration1 mg/ml
ClonalityPolyclonal
Clone Number
ImmunogenSynthesized peptide derived from human protein . at AA range: 420-500
Sequence
SpecificityARSB Polyclonal Antibody detects endogenous levels of protein.
Gene NameARSB
Protein NameArylsulfatase B (ASB) (N-acetylgalactosamine-4-sulfatase) (G4S)
Other Name
SpeciesHuman
Gene ID-1411
UniprotP15848,
Species.1Mouse
Gene ID-2
Uniprot.1P50429,
Species.2Rat
Gene ID-3
Uniprot.2P50430
Organism-4
Gene ID-4
SwissProt-4
BackgroundArylsulfatase 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 targetted 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, Jul 2008],
Cellular LocalizationLysosome . Cell surface .
Tissue ExpressionCerebellum,Colon,Liver,Placenta,
Signaling_pathwayCellular Processes >> Transport and catabolism >> Lysosome
Research Areas>>Glycosaminoglycan degradation; / >>Metabolic pathways; / >>Lysosome
FunctionCatalytic activity:Hydrolysis of the 4-sulfate groups of the N-acetyl-D-galactosamine 4-sulfate units of chondroitin sulfate and dermatan sulfate.,cofactor:Binds 1 calcium ion per subunit.,Disease: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.,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.,PTM: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).,similarity:Belongs to the sulfatase family.,subunit:Monomer.,
RRID
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