Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 1976 Apr;57(4):1036-40.
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

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

J Singh et al. J Clin Invest. 1976 Apr.

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.

PubMed Disclaimer

References

    1. J Biol Chem. 1956 May;220(1):303-6 - PubMed
    1. J Biol Chem. 1949 Nov;181(1):149-51 - PubMed
    1. J Lab Clin Med. 1962 May;59:779-91 - PubMed
    1. Biochem J. 1962 Jul;84:106-10 - PubMed
    1. Biochem J. 1961 Feb;78:312-9 - PubMed

Publication types

MeSH terms