Development of a functional bioassay for arylsulfatase B using the natural substrates of the enzyme

Analytical Biochemistry

Volumn 395, Issue 2, 2009, Pages 144-150

  Pungor Jr , Erno ^a   Hague, Charles M ^a   Chen, Ginger ^a   Lemontt, Jeffrey F ^a   Dvorak Ewell, Melita ^a   Prince, William S ^a  

^a BIOMARIN PHARMACEUTICAL INC   (United States)

Author keywords

Arylsulfatase B; Bioassay; Chondroitin sulfate; Dematan sulfate; LIF CZE; Natural substrate

Indexed keywords

BIOASSAY; CAPILLARY ELECTROPHORESIS; CELL CULTURE; ENZYMES; SULFUR COMPOUNDS;

ARYLSULFATASE; CHONDROITIN SULFATES; DEMATAN SULFATE; LIF-CZE; NATURAL SUBSTRATES;

SUBSTRATES;

CHONDROITIN ABC LYASE; CHONDROITIN SULFATE; DERMATAN SULFATE; DISACCHARIDE; GALSULFASE; GLYCOSAMINOGLYCAN; HYDROLASE; LYSOSOME ENZYME; N ACETYLGALACTOSAMINE 4 SULFATASE; OLIGOSACCHARIDE;

ARTICLE; BIOASSAY; CAPILLARY ZONE ELECTROPHORESIS; CELL TRANSPORT; CONTROLLED STUDY; CULTURE MEDIUM; ENZYME ACTIVITY; ENZYME DEFICIENCY; ENZYME MECHANISM; ENZYME SUBSTRATE; FIBROBLAST CULTURE; FLUORESCENCE ANALYSIS; HUMAN; HUMAN CELL; HYDROLYSIS; IN VITRO STUDY; INCUBATION TIME; MOLECULAR SIZE; PRIORITY JOURNAL; PROTEIN CLEAVAGE; PROTEIN DEPLETION; PROTEIN STRUCTURE;

EID: 70349783746     PISSN: 00032697     EISSN: 10960309     Source Type: Journal    
DOI: 10.1016/j.ab.2009.08.012     Document Type: Article

References (18)

1. Iozzo R.V. Matrix proteoglycans: molecular properties, protein interactions, and role in physiological processes. Physiol. Rev. 71 (1998) 481-539
2. von Figura K., Kresse H., Meinhard U., and Holftrerich D. Effects of anti-microtubular agents on secretion and endocytosis of lysosomal enzymes and of sulphated glycosaminoglycans. Biochem. J. 170 (1978) 313-320
3. Chang P.L., Rosa N.E., and Davidson R.G. Differential assay of arylsulfatase A and B activities: a sensitive method for cultured human cells. Anal. Biochem. 117 (1981) 382-389
4. Hopwood J.J., and Elliott H. Detection of Morquio A syndrome using radiolabelled substrates derived from keratan sulphate for the estimation of galactose 6-sulphate sulphatase. Clin. Sci. 65 (1983) 325-331
5. Hopwood J.J., and Elliott H. Urinary excretion of sulphated N-acetylhexosamines in patients with various mucopolysaccharidosis. Biochem. J. 229 (1985) 579-586
6. Hopwood J.J., Elliott H., Muller J.V., and Saccone G.T.P. Diagnosis of Maroteaux-Lamy syndrome by the use of radiolabelled oligosaccharides as substrates for the determination of arylsulphatase B activity. Biochem. J. 234 (1986) 507-514
7. Anson D.S., Muller V., Bielicki J., Harper G.S., and Hopwood J.J. Overexpression of N-acetylgalactosamine-4-sulphatase induces a multiple sulphatase deficiency in mucopolysaccharidosis-type-VI fibroblasts. Biochem. J. 294 (1993) 657-662
8. Kjellen L., and Lindahl U. Proteoglycans: structures and interactions. Annu. Rev. Biochem. 60 (1991) 443-475
9. Jin W.-D., Jackson C.E., Desnick R.J., and Schuchman E.H. Mucopolysaccharidosis type VI: identification of three mutations in the arylsulfatase B gene of patients with the severe and mild phenotypes provides molecular evidence for genetic heterogeneity. Am. J. Hum. Genet. 50 (1992) 795-800
10. Neufeld E.F., and Muenzer J. The mucopolysaccharidoses. In: Scriver C.R., Beudet A.L., Sly W.S., and Valle D. (Eds). The Metabolic and Molecular Bases of Inherited Disease. seventh ed. (1995), McGraw-Hill, New York 2465-2494
11. U.S. Department of Health and Human Services, ICH Q6B Specifications: Test Procedures and Acceptance Criteria for Biotechnological/Biological Products, U.S. Department of Health and Human Services, Food and Drug Administration, Center for Drug Evaluation and Research, Center for Biologics Evaluation and Research, 1999.
12. Kaplan A., Achord D.T., and Sly W.S. Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts. Proc. Natl. Acad. Sci. USA 74 (1977) 2026-2030
13. Kornfeld S. Trafficking of lysosomal enzymes in normal and disease states. J. Clin. Invest. 77 (1986) 1-6
14. Hille-Rehfeld A. Mannose 6-phosphate receptors in sorting and transport of lysosomal enzymes. Biochim. Biophys. Acta 1241 (1995) 177-194
15. Fuller M., Hopwood J.J., and Anson D.S. Receptor mediated binding of two glycosylation forms of N-acetylgalactosamine-4-sulphatase. Biochim. Biophys. Acta 1406 (1998) 283-290
16. Schmidt B., Selmer T., Ingendoh A., and von Figura K. A novel amino acid modification in sulfatases that is defective in multiple sulfatase deficiency. Cell 82 (1995) 271-278
17. Fuller M., Meikle P.J., and Hopwood J. Glycosaminoglycan degradation fragments in mucopolysaccharidosis I. Glycobiology 14 (2004) 443-450
18. Hoppe W., Rauch U., and Kresse H. Degradation of endocytosed dermatan sulfate proteoglycan in human fibroblasts. J. Biol. Chem. 263 (1988) 5926-5932