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

85 Citations (Scopus)

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

Fingerprint

Mucopolysaccharidosis IV
Sulfatases
Chondroitin Sulfates
Sulfates
Osteochondrodysplasias
Keratan Sulfate
Chondrosarcoma
Chick Embryo
Fibroblasts
Rats
Assays
Skin
Urine
Substrates
Enzymes

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

Cite this

Morquio's syndrome : Deficiency of a chondroitin sulfate N-acetylhexosamine sulfate sulfatase. / Matalon, Reuben; Arbogast, Bradley; Justice, Parvin; Brandt, Ira K.; Dorfman, Albert.

In: Biochemical and Biophysical Research Communications, Vol. 61, No. 2, 27.11.1974, p. 759-765.

Research output: Contribution to journalArticle

Matalon, Reuben ; Arbogast, Bradley ; Justice, Parvin ; Brandt, Ira K. ; Dorfman, Albert. / Morquio's syndrome : Deficiency of a chondroitin sulfate N-acetylhexosamine sulfate sulfatase. In: Biochemical and Biophysical Research Communications. 1974 ; Vol. 61, No. 2. pp. 759-765.
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