Advertisement

Research Article Free access | 10.1172/JCI108345

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

J Singh, N Di Ferrante, P Niebes, and D Tavella

Find articles by Singh, J. in: JCI | PubMed | Google Scholar

Find articles by Di Ferrante, N. in: JCI | PubMed | Google Scholar

Find articles by Niebes, P. in: JCI | PubMed | Google Scholar

Find articles by Tavella, D. in: JCI | PubMed | Google Scholar

Published April 1, 1976 - More info

Published in Volume 57, Issue 4 on April 1, 1976
J Clin Invest. 1976;57(4):1036–1040. https://doi.org/10.1172/JCI108345.
© 1976 The American Society for Clinical Investigation
Published April 1, 1976 - Version history
View PDF
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.

Browse pages

Click on an image below to see the page. View PDF of the complete article

Version history
  • Version 1 (April 1, 1976): No description
Advertisement
Advertisement