Literature DB >> 159821

Urinary acid mucopolysaccharides in multiple sulfatase deficiency (mucosulfatidosis).

Y Eto, S Numaguchi, T Handa.   

Abstract

Urinary acid mucopolysaccharides (AMPS) excretion was investigated in a Japanese case with Multiple Sulfatase Deficiency (MSD) (Mucosulfatidosis). The patient excreted AMPS 4 to 5 times more (as carbazoluronic acid) than controls. The cellulose acetate gel electrophoresis clearly indicated two major AMPS which co-migrated with heparan sulfate and chondroitin sulfate A/C. Enzymic digestion with chondroitinase AC and ABC, and by testicular hyaluronidase plus amino sugar analysis also confirmed that our case excreted heparan sulfate and chondroitin sulfate A/C. These findings suggest that there are heterogeneities of urinary AMPS excretion among cases with MSD.

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Year:  1979        PMID： 159821     DOI： 10.1007/bf00442437

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN： 0340-6199            Impact factor:   3.183


  11 in total

1.  A method for the separation of acid mucopolysaccharides: its application to the isolation of heparin from the skin of rats.

Authors:  S SCHILLER; G A SLOVER; A DORFMAN
Journal:  J Biol Chem       Date:  1961-04       Impact factor: 5.157

2.  A new specific color reaction of hexuronic acids.

Authors:  Z DISCHE
Journal:  J Biol Chem       Date:  1947-01       Impact factor: 5.157

3.  Enzymatic methods for the determination of small quantities of isomeric chondroitin sulfates.

Authors:  H Saito; T Yamagata; S Suzuki
Journal:  J Biol Chem       Date:  1968-04-10       Impact factor: 5.157

4.  Structure of dermatan sulfate. I. Degradation by testicular hyaluronidase.

Authors:  L A Fransson; L Rodén
Journal:  J Biol Chem       Date:  1967-09-25       Impact factor: 5.157

5.  Multiple sulfatase deficiencies in cultured skin fibroblasts. Occurrence in patients with a variant form of metachromatic leukodystrophy.

Authors:  Y Eto; U N Wiesmann; J H Carson; N N Herschkowitz
Journal:  Arch Neurol       Date:  1974-02

6.  [Combination of metachromatic leucodystrophy and mucopolysaccharidosis: a disease entity (mucosulfatidosis)].

Authors:  S Rampini; W Isler; K Baerlocher; A Bischoff; J Ulrich; H J Plüss
Journal:  Helv Paediatr Acta       Date:  1970-11

7.  Enzymic studies of sulphatases in tissues of the normal human and in metachromatic leukodystrophy with multiple sulphatase deficiencies: arylsulphatases A, B and C, cerebroside sulphatase, psychosine sulphatase and steroid sulphatases.

Authors:  Y Eto; S Rampini; U Wiesmann; N N Herschkowitz
Journal:  J Neurochem       Date:  1974-12       Impact factor: 5.372

8.  Metachromatic leukodystrophy (MLD). VII. Elevated sulfated acid polysaccharide levels in urine and postmortem tissues.

Authors:  M Bischel; J Austin; M Kemeny
Journal:  Arch Neurol       Date:  1966-07

9.  Metachromatic form of diffuse cerebral sclerosis. V. The nature and significance of low sulfatase activity: a controlled study of brain, liver and kidney in four patients with metachromatic leukodystrophy (MLD).

Authors:  J Austin; D Armstrong; L Shearer
Journal:  Arch Neurol       Date:  1965-12

10.  Arylsulfatase B deficiency in Maroteaux-Lamy syndrome cultured fibroblasts.

Authors:  A L Fluharty; R L Stevens; D L Sanders; H Kihara
Journal:  Biochem Biophys Res Commun       Date:  1974-07-24       Impact factor: 3.575
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  2 in total

1.  Multiple sulfatase deficiency with a novel biochemical presentation.

Authors:  G Constantopoulos
Journal:  Eur J Pediatr       Date:  1988-08       Impact factor: 3.183

2.  Increased urinary excretion of acid mucopolysaccharides and glycopeptides in hypothyroidism following thyroid hormone therapy.

Authors:  T Tokoro; Y Eto
Journal:  Eur J Pediatr       Date:  1985-05       Impact factor: 3.183
  2 in total

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