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

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

doi:10.1016/0006-291X(73)90809-7

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

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

Research output: Contribution to journal › Article › peer-review

55 Scopus citations

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 language	English (US)
Pages (from-to)	1125-1132
Number of pages	8
Journal	Biochemical and Biophysical Research Communications
Volume	54
Issue number	3
DOIs	https://doi.org/10.1016/0006-291X(73)90809-7
State	Published - Oct 1 1973
Externally published	Yes

ASJC Scopus subject areas

Biophysics
Biochemistry
Molecular Biology
Cell Biology

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title = "Hunter's syndrome: A deficiency of L-idurono-sulfate sulfatase",

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.",

author = "Ingrid Sj{\"o}berg and Fransson, \{Lars Ake\} and Reuben Matalon and Albert Dorfman",

year = "1973",

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doi = "10.1016/0006-291X(73)90809-7",

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T2 - A deficiency of L-idurono-sulfate sulfatase

AU - Sjöberg, Ingrid

AU - Fransson, Lars Ake

AU - Matalon, Reuben

AU - Dorfman, Albert

PY - 1973/10/1

Y1 - 1973/10/1

N2 - [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.

AB - [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.

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ER -

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

Abstract

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