Rabbit anti-Human ARSB Polyclonal Antibody | anti-ARSB antibody

ARSB, CT (Arylsulfatase B, ASB, N-acetylgalactosamine-4-sulfatase, G4S) (PE)

Cat. Num. AAA6464186

• Gene Names: ARSB; ASB; G4S; MPS6
• Reactivity: Human
• Applications: Immunohistochemistry, ELISA, Western Blot
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: ARSB; Polyclonal Antibody; CT (Arylsulfatase B; ASB; N-acetylgalactosamine-4-sulfatase; G4S) (PE); anti-ARSB antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human ARSB.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).

• Applicable Applications for anti-ARSB antibody: Immunohistochemistry (IHC), ELISA (EIA), Western Blot (WB)
• Application Notes: IHC: 1:10-1:50; ELISA: 1:1,000; WB: 1:50-1:100; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the C-terminal region of human ARSB (KLH).
• Conjugate: PE
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of ARSB antibody (C-term) in HepG2 cell line lysates (35ug/lane). ARSB (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human lung carcinoma tissue reacted with ARSB antibody (C-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Product Categories/Family for anti-ARSB antibody

Antibodies; Disease Markers

References

1. Peters C., Schmidt B.J. Biol. Chem. 265:3374-3381(1990). 2. Modaressi S., Rupp K.Biol. Chem. Hoppe-Seyler 374:327-335(1993). 3. Kobayashi T., Honke K.Biochim. Biophys. Acta 1159:243-247(1992).

NCBI and Uniprot Product Information

• NCBI GI #: 38569405
• NCBI GeneID: 411
• NCBI Accession #: NP_000037
• NCBI GenBank Nucleotide #: NM_000046.3
• UniProt Accession #: P15848; Q8N322; Q9UDI9; B2RC20
• Molecular Weight: Predicted: 59 kDa; ; Observed: 57kDa
• 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; N-acetylgalactosamine-4-sulfatase
• 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 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]

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