Abstract
Upon incubation of keratan [35S]sulphate with normal fibroblasts both [35S]sulphate and N-acetylglucosamine 6-[35S]sulphate are liberated. From the products obtained after digestion with various mutant fibroblasts and with purified N-acetylgalactosamine 6-sulphate sulphatase we suggest that (i) [35S]sulphate is released almost exclusively from galactose 6-sulphate residues; (ii) N-acetylgalactosamine 6-sulphate sulphatase exhibits galactose 6-sulphate sulphatase activity; (iii) both sulphatase activities are deficient in Morquio disease type A.
Full text
PDF



Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Basner R., Kresse H., von Figura K. N-Acetylglucosamine-6-sulfate sulfatase from human urine. J Biol Chem. 1979 Feb 25;254(4):1151–1158. [PubMed] [Google Scholar]
- Di Ferrante N., Ginsberg L. C., Donnelly P. V., Di Ferrante D. T., Caskey C. T. Deficiencies of glucosamine-6-sulfate or galactosamine-6-sulfate sulfatases are responsible for different mucopolysaccharidoses. Science. 1978 Jan 6;199(4324):79–81. doi: 10.1126/science.199.4324.79. [DOI] [PubMed] [Google Scholar]
- Di Ferrante N. N-acetylglucosamine-6-sulfate sulfatase deficiency reconsidered. Science. 1980 Oct;210(4468):448–448. doi: 10.1126/science.6776626. [DOI] [PubMed] [Google Scholar]
- Glössl J., Kresse H. A sensitive procedure for the diagnosis of N-acetyl-galactosamine-6-sulfate sulfatase deficiency in classical Morquio's disease. Clin Chim Acta. 1978 Aug 15;88(1):111–119. doi: 10.1016/0009-8981(78)90157-2. [DOI] [PubMed] [Google Scholar]
- Glössl J., Truppe W., Kresse H. Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta. Biochem J. 1979 Jul 1;181(1):37–46. doi: 10.1042/bj1810037. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Hasilik A., Neufeld E. F. Biosynthesis of lysosomal enzymes in fibroblasts. Synthesis as precursors of higher molecular weight. J Biol Chem. 1980 May 25;255(10):4937–4945. [PubMed] [Google Scholar]
- Horwitz A. L., Dorfman A. The enzymic defect in Morquio's disease: the specificity of N-acetylhexosamine sulfatases. Biochem Biophys Res Commun. 1978 Feb 28;80(4):819–825. doi: 10.1016/0006-291x(78)91318-9. [DOI] [PubMed] [Google Scholar]
- Kresse H., Paschke E., von Figura K., Gilberg W., Fuchs W. Sanfilippo disease type D: deficiency of N-acetylglucosamine-6-sulfate sulfatase required for heparan sulfate degradation. Proc Natl Acad Sci U S A. 1980 Nov;77(11):6822–6826. doi: 10.1073/pnas.77.11.6822. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Ludolph T., Paschke E., Glössl J., Kresse H. Degradation of keratan sulphate by beta-N-acetylhexosaminidases A and B. Biochem J. 1981 Mar 1;193(3):811–818. doi: 10.1042/bj1930811. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Matalon R., Arbogast B., Justice P., Brandt I. K., Dorfman A. Morquio's syndrome: deficiency of a chondroitin sulfate N-acetylhexosamine sulfate sulfatase. Biochem Biophys Res Commun. 1974 Nov 27;61(2):759–765. doi: 10.1016/0006-291x(74)91022-5. [DOI] [PubMed] [Google Scholar]
- Minami R., Abo K., Kudoh T., Tsugawa S., Oyanagi K., Nakao T. Identification of keratan sulfate in liver affected by Morquio syndrome. Clin Chim Acta. 1979 Apr 16;93(2):207–213. doi: 10.1016/0009-8981(79)90090-1. [DOI] [PubMed] [Google Scholar]
- PEDRINI V., LENNZI L., ZAMBOTTI V. Isolation and identification of keratosulphate in urine of patients affected by Morquio-Ullrich disease. Proc Soc Exp Biol Med. 1962 Aug-Sep;110:847–849. doi: 10.3181/00379727-110-27668. [DOI] [PubMed] [Google Scholar]
- REISSIG J. L., STORMINGER J. L., LELOIR L. F. A modified colorimetric method for the estimation of N-acetylamino sugars. J Biol Chem. 1955 Dec;217(2):959–966. [PubMed] [Google Scholar]
- Singh J., Di Ferrante N., Niebes P., Tavella D. N-acetylgalactosamine-6-sulfate sulfatase in man. Absence of the enzyme in Morquio disease. J Clin Invest. 1976 Apr;57(4):1036–1040. doi: 10.1172/JCI108345. [DOI] [PMC free article] [PubMed] [Google Scholar]