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

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

Research output: Contribution to journalArticle

51 Citations (Scopus)

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
Externally publishedYes

Fingerprint

Mucopolysaccharidosis II
Sulfatases
Dermatan Sulfate
Sulfates
Chondroitinases and Chondroitin Lyases
Paper Electrophoresis
Disaccharides
Ion Exchange Chromatography
Fibroblasts
Chromatography
Electrophoresis
Hydrolysis
Ion exchange
Oxidation
Acids

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

Cite this

Hunter's syndrome : A deficiency of L-idurono-sulfate sulfatase. / Sjöberg, Ingrid; Fransson, Lars Ake; Matalon, Reuben; Dorfman, Albert.

In: Biochemical and Biophysical Research Communications, Vol. 54, No. 3, 01.10.1973, p. 1125-1132.

Research output: Contribution to journalArticle

Sjöberg, Ingrid ; Fransson, Lars Ake ; Matalon, Reuben ; Dorfman, Albert. / Hunter's syndrome : A deficiency of L-idurono-sulfate sulfatase. In: Biochemical and Biophysical Research Communications. 1973 ; Vol. 54, No. 3. pp. 1125-1132.
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