Goat ARSB / Arylsulfatase B Polyclonal Antibody | anti-ARSB antibody

Goat Polyclonal to Human ARSB / Arylsulfatase B

Cat. Num. AAA241969

• Gene Names: ARSB; ASB; G4S; MPS6
• Reactivity: Gibbon, Gorilla, Human, Monkey
• Applications: Immunohistochemistry, Western Blot, ELISA
• Purity: Immunoaffinity Purified
• Synonyms: ARSB / Arylsulfatase B; Polyclonal Antibody; Goat Polyclonal to Human ARSB / Arylsulfatase B; Anti-ARSB / Arylsulfatase B Antibody (Internal) IHC-plus; ARSB; Arylsulfatase B; ASB; G4S; MPS6; Human ARSB; anti-ARSB antibody

• Host: Goat
• Reactivity: Gibbon, Gorilla, Human, Monkey
• Clonality: Polyclonal
• Specificity: Human ARSB. This antibody is expected to recognise both reported isoforms (NP_000037.2; NP_942002.1).
• Purity/Purification: Immunoaffinity Purified
• Form/Format: Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide
• Concentration: 0.5 mg/ml (varies by lot)
• Sequence Length: 533

• Applicable Applications for anti-ARSB antibody: Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)
• Application Notes: ELISA (1:64000), IHC-P (5 ug/ml), WB (0.03 - 0.1 ug/ml); Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary anti...
• Target Species: Human
• Immunogen Description: Synthetic peptide C-KLARGHTNGTKPLD from an internal region of human ARSB (NP_000037.2; NP_942002.1). Percent identity by BLAST analysis: Human, Gorilla, Gibbon, Marmoset (100%); Elephant (93%); Monkey, Bovine, Bat, Cat, Panda, Horse, Chicken (86%).
• Immunogen Type: Synthetic peptide
• Immunogen: ARSB / Arylsulfatase B antibody was raised against synthetic peptide C-KLARGHTNGTKPLD from an internal region of human ARSB (NP_000037.2; NP_942002.1). Percent identity by BLAST analysis: Human, Gorilla, Gibbon, Marmoset (100%); Elephant (93%); Monkey, Bovine, Bat, Cat, Panda, Horse, Chicken (86%).
• Antigen Modification: Internal
• Preparation and Storage: Store at -20 degree C. Minimize freezing and thawing.

Immunohistochemistry (IHC)

(Anti-ARSB antibody IHC of human skeletal muscle. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.)

Western Blot (WB)

(Antibody (0.03 ug/ml) staining of Human Heart lysate (35 ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

Related Product Information for anti-ARSB antibody

The arylsulfatase B homodimer hydrolyzes sulfate groups of N-Acetyl-D-galactosamine, chondriotin sulfate, and dermatan sulfate. The protein is targetted to the lysozyme. Defects in this gene cause Maroteaux-Lamy syndrome.

NCBI and Uniprot Product Information

• NCBI GI #: 38569405
• NCBI GeneID: 411
• NCBI Accession #: NP_000037.2
• NCBI GenBank Nucleotide #: NM_000046.3
• UniProt Accession #: P15848; Q8N322; Q9UDI9; B2RC20
• Molecular Weight: 45,996 Da
• 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: Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; EC 3.1.6.12

Chromosomal Location of Human Ortholog: 5q14.1

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

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

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

Disease: Mucopolysaccharidosis Type Vi