Arylsulfatase B
Title: Arylsulfatase B
CAS Registry Number: 55354-43-3
CAS Name: Acetylgalactosamine 4-sulfatase
Additional Names: sulfatase B; EC
Literature References: Lysosomal enzyme that hydrolyzes the 4-sulfate ester of N-acetylgalactosamine at the nonreducing terminus of dermatan sulfate and chondroitin-4-sulfate. Genetic deficiency of the enzyme results in the lysosomal storage disorder, mucopolysaccharidosis-VI (MPS-VI), also known as Maroteaux-Lamy syndrome. Purification from ox liver: A. B. Roy, Biochem. J. 57, 30 (1954); from human liver: K. S. Dodgson, C. H. Wynn, ibid. 68, 387 (1958). Identification of role in disease: D. A. Stumpf, J. H. Austin, Trans. Amer. Neurol. Assoc. 97, 29 (1972). Identification of natural substrate: A. A. Farooqui, Experientia 32, 1242 (1976). Prepn of recombinant human form and uptake by human fibroblast cells: D. S. Anson et al., Biochem. J. 284, 789 (1992). Molecular structure and characterization of active site: C. S. Bond et al., Structure 5, 277 (1997).
Derivative Type: Galsulfase
CAS Registry Number: 552858-79-4
CAS Name: Acetylgalactosamine 4-sulfatase (human CSL4S-342 cell)
Additional Names: aryplase; rhASB
Manufacturers' Codes: BM-102
Trademarks: Naglazyme (BioMarin)
Literature References: Human arylsulfatase B produced by recombinant DNA technology in Chinese hamster ovary cells. Glycoprotein comprised of 495 amino acids; mol wt ~56 kDa. Prepn: C. M. Starr, WO 0183722 (2001 to BioMarin). Clinical evaluation in children with MPS-VI: P. Harmatz et al., J. Pediatr. 144, 574 (2004). Review: G. Yogalingam, Curr. Opin. Invest. Drugs 5, 1111-1120 (2004).
Therap-Cat: Enzyme replacement therapy for mucopolysaccharidosis-VI.
Keywords: Enzyme Replacement Therapy.

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