Arylsulfatase B, Polyclonal Antibody

ARSB; arylsulfatase B; ASB; G4S; MPS6; N-acetylgalactosamine-4-sulfatase; ARSB antibody; arylsulfatase B antibody; ASB antibody; G4S antibody; MPS6 antibody; N-acetylgalactosamine-4-sulfatase antibody; Arylsulfatase B; Arylsulfatase B

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul (lot specific)

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-ARSB antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Peptide ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Peptide ELISA: Antibody detection limit dilution 1: 64000.
Immunohistochemistry: In paraffin embedded Human Skeletal Muscle shows striated pattern staining of majority of muscular fibres. Recommended concentration, 5-10ug/ml.

(0. 03ug/ml) staining of Human Heart lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.

(5ug/ml) staining of paraffin embedded Human Skeletal Muscle. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.

45,996 Da

arylsulfatase B

arylsulfatase B

Arylsulfatase B

ASB; G4S  [Similar Products]

ARSB_HUMAN

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]

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: Hydrolase; EC 3.1.6.12; Glycan Metabolism - glycosaminoglycan degradation

Chromosomal Location of Human Ortholog: 5q14.1

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

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

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

Disease: Mucopolysaccharidosis Type Vi

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