Mouse ARSB Monoclonal Antibody | anti-ARSB antibody

ARSB (arylsulfatase B, ASB, G4S, MPS6) (FITC)

Cat. Num. AAA6178437

• Gene Names: ARSB; ASB; G4S; MPS6
• Applications: Western Blot
• Purity: Purified
• Synonyms: ARSB; Monoclonal Antibody; ARSB (arylsulfatase B; ASB; G4S; MPS6) (FITC); arylsulfatase B; MPS6; anti-ARSB antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG1,k
• Clone Number: 2G6
• Specificity: Recognizes ARSB.
• Purity/Purification: Purified
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).
• Sequence Length: 413

• Applicable Applications for anti-ARSB antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: ARSB (NP_942002, 166aa-265aa) partial recombinant protein with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: FGYLLGSEDYYSHERCTLIDALNVTRCALDFRDGEEVATGYKNMYSTNIFTKRAIALITNHPPEKPLFLYLALQSVHEPLQVPEEYLKPYDFIQDKNRHH
• Conjugate: FITC
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer.; ; FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Testing Data

(Detection limit for recombinant GST tagged ARSB is 1 ng/ml as a capture antibody.)

Related Product Information for anti-ARSB antibody

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]

Product Categories/Family for anti-ARSB antibody

Antibodies; Disease Markers

NCBI and Uniprot Product Information

• NCBI GI #: 38569407
• NCBI GeneID: 411
• NCBI Accession #: NP_942002
• NCBI GenBank Nucleotide #: NP_942002.1
• UniProt Accession #: P15848
• NCBI Official Full Name: arylsulfatase B isoform 2
• 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