Morquio's syndrome: Deficiency of a chondroitin sulfate N-acetylhexosamine sulfate sulfatase

Reuben Matalon, Bradley Arbogast, Parvin Justice, Ira K. Brandt, Albert Dorfman

Research output: Contribution to journalArticle

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Abstract

The Morquio syndrome is a spondyloepiphyseal dysplasia characterized by excretion in urine of excessive amounts of keratan sulfate and chondroitin sulfate. To investigate the enzymic basis of this disease, assays for sulfatase were performed using chick embryo chondroitin sulfate and rat chondrosarcoma chondroitin 4-sulfate as substrates. The data obtained, using skin fibroblasts as an enzyme source, indicate that Morquio's syndrome is a deficiency of chondroitin sulfate N-acetylhexosamine sulfate sulfatase.

Original languageEnglish (US)
Pages (from-to)759-765
Number of pages7
JournalBiochemical and Biophysical Research Communications
Volume61
Issue number2
DOIs
StatePublished - Nov 27 1974
Externally publishedYes

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ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

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