Deficiency of chondroitin sulfate N-acetylgalactosamine 4-sulfate sulfatase in Maroteaux-Lamy syndrome

Reuben Matalon, Bradley Arbogast, Albert Dorfman

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The Maroteaux-Lamy syndrome (Mucopolysaccharidosis VI) is characterized by excessive excretion in urine and deposition in tissue of dermatan sulfate. To investigate the enzymic basis of this disease [35S]chondroitin 4-sulfate was prepared utilizing a rat chondrosarcoma. Extracts of fibroblasts derived from patients with this syndrome showed a marked diminution of release of 35SO4 both from [35S]chondroitin 4-SO4 and from a heptasaccharide derived from this substance. Since there is a marked deficiency of arylsulfatase B activity in this disease, it is concluded that natural substrate of arylsulfatase B is N-acetylgalactosamine 4-SO4 in chondroitin 4-SO4 and dermatan sulfate.

Original languageEnglish (US)
Pages (from-to)1450-1457
Number of pages8
JournalBiochemical and Biophysical Research Communications
Issue number4
StatePublished - Dec 23 1974
Externally publishedYes


ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

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