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

Reuben Matalon; Bradley Arbogast; Albert Dorfman

doi:10.1016/S0006-291X(74)80446-8

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

Reuben Matalon, Bradley Arbogast, Albert Dorfman

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Abstract

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 language	English (US)
Pages (from-to)	1450-1457
Number of pages	8
Journal	Biochemical and Biophysical Research Communications
Volume	61
Issue number	4
DOIs	https://doi.org/10.1016/S0006-291X(74)80446-8
State	Published - Dec 23 1974
Externally published	Yes

ASJC Scopus subject areas

Biophysics
Biochemistry
Molecular Biology
Cell Biology

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10.1016/S0006-291X(74)80446-8

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title = "Deficiency of chondroitin sulfate N-acetylgalactosamine 4-sulfate sulfatase in Maroteaux-Lamy syndrome",

abstract = "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.",

author = "Reuben Matalon and Bradley Arbogast and Albert Dorfman",

note = "Funding Information: This work was supported by USPHS Grants AM-05996, HD-04583 and a Funding Information: grant from the Chicago Heart Association.",

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T1 - Deficiency of chondroitin sulfate N-acetylgalactosamine 4-sulfate sulfatase in Maroteaux-Lamy syndrome

AU - Matalon, Reuben

AU - Arbogast, Bradley

AU - Dorfman, Albert

N1 - Funding Information: This work was supported by USPHS Grants AM-05996, HD-04583 and a Funding Information: grant from the Chicago Heart Association.

PY - 1974/12/23

Y1 - 1974/12/23

N2 - 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.

AB - 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.

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

Abstract

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