Arylsulfatase B Antibody (2G6): sc-517158

Arylsulfatase B Antibody (2G6) is a mouse monoclonal IgG1 kappa light chain antibody that detects Arylsulfatase B protein of human origin by western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). Anti-Arylsulfatase B antibody (2G6) is available as a non-conjugated monoclonal isotype antibody. Arylsulfatase B, also known as ARSB, ASB, MPS6, or G4S, is a 533 amino acid lysosomal protein that plays a crucial role in the hydrolysis of C4-sulfate groups from various substrates, including N-Acetyl-D-galactosamine, dermatan sulfate, and chondroitin sulfate, utilizing calcium as a cofactor. This enzymatic activity is vital for proper lysosomal degradation of glycosaminoglycans, and any defects in the gene encoding Arylsulfatase B can lead to severe lysosomal storage disorders such as mucopolysaccharidosis type 6 (MPS6) and multiple sulfatase deficiency (MSD). MPS6 is characterized by a range of debilitating symptoms, including short stature, joint stiffness, skeletal malformations, corneal clouding, hepatosplenomegaly, and cardiac issues, while MSD results in reduced activity of all known sulfatases and is often associated with additional complications such as chondrodysplasia punctata and neurological deterioration. Multiple isoforms of Arylsulfatase B, arising from alternative splicing events, further underscore the complexity and importance of this protein in human health.

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Arylsulfatase B Antibody (2G6) References:

1. Mucopolysaccharidosis type VI: Structural and clinical implications of mutations in N-acetylgalactosamine-4-sulfatase.  |  Litjens, T. and Hopwood, JJ. 2001. Hum Mutat. 18: 282-95. PMID: 11668612
2. Increased arylsulfatase B activity in cystic fibrosis cells following correction of CFTR.  |  Bhattacharyya, S., et al. 2007. Clin Chim Acta. 380: 122-7. PMID: 17324393
3. Distinct effects of N-acetylgalactosamine-4-sulfatase and galactose-6-sulfatase expression on chondroitin sulfates.  |  Bhattacharyya, S., et al. 2008. J Biol Chem. 283: 9523-30. PMID: 18285341
4. Maroteaux-Lamy syndrome: functional characterization of pathogenic mutations and polymorphisms in the arylsulfatase B gene.  |  Garrido, E., et al. 2008. Mol Genet Metab. 94: 305-12. PMID: 18406185
5. Human N-acetylgalactosamine-4-sulphatase: protein maturation and isolation of genomic clones.  |  Litjens, T., et al. 1991. Biochem Int. 24: 209-15. PMID: 1930244
6. Structure of the human arylsulfatase B gene.  |  Modaressi, S., et al. 1993. Biol Chem Hoppe Seyler. 374: 327-35. PMID: 7687847
7. Four novel mutant alleles of the arylsulfatase B gene in two patients with intermediate form of mucopolysaccharidosis VI (Maroteaux-Lamy syndrome).  |  Voskoboeva, E., et al. 1994. Hum Genet. 93: 259-64. PMID: 8125475
8. Structure of a human lysosomal sulfatase.  |  Bond, CS., et al. 1997. Structure. 5: 277-89. PMID: 9032078