Hunter's syndrome: A deficiency of L-idurono-sulfate sulfatase

Ingrid Sjöberg, Lars Ake Fransson, Reuben Matalon, Albert Dorfman

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Abstract

[35SO4] Dermatan sulfate, isolated from normal Hurler and Hunter fibroblasts was degraded by chondroitinase A, B, C to yield mono-and disaccharides. The products were separated by ion exchange chromatography and those arising from the non-reducing terminus were characterized by paper electrophoresis. The position of sulfate substituents was established by periodate oxidation and partial acid hydrolysis. Normal dermatan sulfate terminates with GalN-SO4 whereas IdUA-SO4 was a prominent terminus in Hunter dermatan sulfate but not in Hurler dermatan sulfate. It is concluded that Hunter's syndrome is due to a deficiency of L-idurono-sulfate sulfatase.

Original languageEnglish (US)
Pages (from-to)1125-1132
Number of pages8
JournalBiochemical and Biophysical Research Communications
Volume54
Issue number3
DOIs
StatePublished - Oct 1 1973

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ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Molecular Biology
  • Cell Biology

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