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. 1976 Apr;57(4):1036–1040. doi: 10.1172/JCI108345

N-acetylgalactosamine-6-sulfate sulfatase in man. Absence of the enzyme in Morquio disease.

J Singh, N Di Ferrante, P Niebes, D Tavella
PMCID: PMC436747  PMID: 820716

Abstract

Human N-acetylgalactosamine-6-sulfate sulfatase (6-sulfatase) activity is measured by using as a substrate a sulfated tetrasaccharide obtained by digesting purified chondroitin-6-sulfate (C-6-S) with testicular hyaluronidase. The amount of inorganic sulfate released is measured turbidimetrically. The enzyme from human kidney has a pH optimum of 4.8; its activity is augmented by low levels of NaCl and inhibited by phosphate and high levels of NaCl. Free glucuronate, acetylgalactosamine, inorganic sulfate, polymeric C-6-S, or tetrasaccharide obtained from chondroitin-4-sulfate do not affect the enzyme activity. The method may be used for the diagnosis of Morquio disease since extracts of Morquio fibroblasts are devoid of 6-sulfatase activity.

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Selected References

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  1. Agogbua S. I., Wynn C. H. The application of affinity chromatography to the arylsulphatase B of human liver and to other arylsulphatases. Biochem Soc Trans. 1975;3(3):405–408. doi: 10.1042/bst0030405. [DOI] [PubMed] [Google Scholar]
  2. Constantopoulos G., Dekaban A. S., Carroll W. R. Determination of molecular weight distribution of acid mucopolysaccharides by sephadex gel filtration. Anal Biochem. 1969 Oct 1;31(1):59–70. doi: 10.1016/0003-2697(69)90241-3. [DOI] [PubMed] [Google Scholar]
  3. DI FERRANTE N. Turbidimetric measurement of acid mucopolysaccharides and hyaluronidase activity. J Biol Chem. 1956 May;220(1):303–306. [PubMed] [Google Scholar]
  4. DODGSON K. S. Determination of inorganic sulphate in studies on the enzymic and non-enzymic hydrolysis of carbohydrate and other sulphate esters. Biochem J. 1961 Feb;78:312–319. doi: 10.1042/bj0780312. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. DODGSON K. S., PRICE R. G. A note on the determination of the ester sulphate content of sulphated polysaccharides. Biochem J. 1962 Jul;84:106–110. doi: 10.1042/bj0840106. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Di Ferrante N., Nichols B. L., Donnelly P. V., Neri G., Hrgovcic R., Berglund R. K. Induced degradation of glycosaminoglycans in Hurler's and Hunter's syndromes by plasma infusion. Proc Natl Acad Sci U S A. 1971 Feb;68(2):303–307. doi: 10.1073/pnas.68.2.303. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. FALLON H. J., FREI E., 3rd, DAVIDSON J. D., TRIER J. S., BURK D. Leukocyte preparations from human blood: evaluation of their morphologic and metabolic state. J Lab Clin Med. 1962 May;59:779–791. [PubMed] [Google Scholar]
  8. Fluharty A. L., Stevens R. L., Fung D., Peak S., Kihara H. Uridine diphospho-N-acetylgalactosamine-4-sulfate sulfohydrolase activity of human arylsulfatase B and its deficiency in the Maroteaux-Lamy syndrome. Biochem Biophys Res Commun. 1975 Jan 2;64(3):955–962. doi: 10.1016/0006-291x(75)90140-0. [DOI] [PubMed] [Google Scholar]
  9. Gatt R., Berman E. R. A rapid procedure for the estimation of amino sugars on a micro scale. Anal Biochem. 1966 Apr;15(1):167–171. doi: 10.1016/0003-2697(66)90262-4. [DOI] [PubMed] [Google Scholar]
  10. LOWRY O. H., ROSEBROUGH N. J., FARR A. L., RANDALL R. J. Protein measurement with the Folin phenol reagent. J Biol Chem. 1951 Nov;193(1):265–275. [PubMed] [Google Scholar]
  11. Nakashima Y., Ferrante N. D., Jackson R. L., Pownall H. J. The interaction of human plasma glycoaminoglycans with plasma lipoproteins. J Biol Chem. 1975 Jul 25;250(14):5386–5392. [PubMed] [Google Scholar]
  12. PARK J. T., JOHNSON M. J. A submicrodetermination of glucose. J Biol Chem. 1949 Nov;181(1):149–151. [PubMed] [Google Scholar]
  13. Saito H., Yamagata T., Suzuki S. Enzymatic methods for the determination of small quantities of isomeric chondroitin sulfates. J Biol Chem. 1968 Apr 10;243(7):1536–1542. [PubMed] [Google Scholar]
  14. Singh J., Donnelly P. V., Di Ferrante N., Nichols B. L., Niebes P. Sanfilippo disease: differentiation of types A and B by an analytical method. J Lab Clin Med. 1974 Sep;84(3):438–450. [PubMed] [Google Scholar]
  15. Singh J., Niebes P., Di Ferrante N. A method for alpha-L-iduronidase assay. FEBS Lett. 1974 Sep 1;45(1):248–251. doi: 10.1016/0014-5793(74)80854-9. [DOI] [PubMed] [Google Scholar]
  16. Yamagata T., Saito H., Habuchi O., Suzuki S. Purification and properties of bacterial chondroitinases and chondrosulfatases. J Biol Chem. 1968 Apr 10;243(7):1523–1535. [PubMed] [Google Scholar]

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