Activities of N-acetylgalactosamine-6-sulfate sulfatase in liver from two sisters with morquio syndrome

Tohoku J Exp Med. 1980 May;131(1):53-7. doi: 10.1620/tjem.131.53.

Abstract

A 6-sulfated tetrasaccharide obtained by digesting chondroitin-6-sulfate with testicular hyaluronidase was used as a substrate for the determination of N-acetylgalactosamine-6-sulfate sulfatase activity. The activity was not detected in liver obtained from the elder sister with clinically classic Morquio syndrome and 4.7% of the control in liver from the younger sister with the same disease.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Chondroitinases and Chondroitin Lyases / metabolism*
  • Chondroitinsulfatases / metabolism*
  • Female
  • Humans
  • Liver / enzymology*
  • Mucopolysaccharidosis IV / enzymology*
  • Mucopolysaccharidosis IV / genetics

Substances

  • Chondroitinsulfatases
  • Chondroitinases and Chondroitin Lyases