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

ARSB Rabbit pAb

Cat. Num. AAA4760682

• Gene Names: ARSB; ASB; G4S; MPS6
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: Affinity purified
• Synonyms: ARSB; Polyclonal Antibody; ARSB Rabbit pAb; arylsulfatase B; ASB; G4S; MPS6; anti-ARSB antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Affinity purified
• Form/Format: pH7.4 PBS, 0.05% NaN3, 40% Glycerol
• Concentration: 0.7mg/ml (varies by lot)

• Applicable Applications for anti-ARSB antibody: Western Blot (WB), Immunohistochemistry-Paraffin (IHC-P)
• Application Notes: WB: 1:200-1:1000; IHC-P: 1:50-1:200
• Immunogen: Synthetic peptide of human ARSB
• Conjugation: Unconjugated
• Modification: Unmodified
• Preparation and Storage: Store at 4 degree C for short term. Store at -20 degree C for long term. Avoid freeze/thaw cycle.

Western Blot (WB)

(Western Blot analysis of ARSB in Human fetal liver tissue using ARSB Rabbit pAb at dilution 1/340)

Immunohistochemistry (IHC)

(Immunohistochemistry of ARSB in paraffin-embedded Human gasrtic cancer tissue using ARSB Rabbit pAb at dilution 1/50. Predicted cell location: Cytoplasm)

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.

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