Literature DB >> 6789675

The correction of Hunter fibroblasts by exogenous iduronate sulfate sulfatase: biochemical and ultrastructural studies.

R Eliahu, E Sekeles, R Cohen, G Bach.   

Abstract

The exogenous addition of iduronate sulfate sulfatase to cultured fibroblasts of Hunter patients resulted in a full correction of the metabolic defect as demonstrated by chemical and ultrastructural analyses. As little as 25% of the normal fibroblasts' enzyme levels were sufficient for this correction. The half-disappearance time of the internalized enzyme was 3-4 days. Prolonged incubation of corrected cells resulted in a gradual reaccumulation of mucopolysaccharides.

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Year:  1981        PMID: 6789675      PMCID: PMC1685092     

Source DB:  PubMed          Journal:  Am J Hum Genet        ISSN: 0002-9297            Impact factor:   11.025


  24 in total

1.  A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.

Authors:  S Hickman; E F Neufeld
Journal:  Biochem Biophys Res Commun       Date:  1972-11-15       Impact factor: 3.575

2.  Hurler and Hunter syndromes: mutual correction of the defect in cultured fibroblasts.

Authors:  J C Fratantoni; C W Hall; E F Neufeld
Journal:  Science       Date:  1968-11-01       Impact factor: 47.728

3.  The defect in Hurler's and Hunter's syndromes: faulty degradation of mucopolysaccharide.

Authors:  J C Fratantoni; C W Hall; E F Neufeld
Journal:  Proc Natl Acad Sci U S A       Date:  1968-06       Impact factor: 11.205

4.  Lipid accumulation and acid lipase deficiency in fibroblasts from a family with Wolman's disease, and their apparent correction in vitro.

Authors:  E C Kyriakides; B Paul; J A Balint
Journal:  J Lab Clin Med       Date:  1972-12

5.  Corrective factors for inborn errors of mucopolysaccharide metabolism.

Authors:  E F Neufeld; M J Cantz
Journal:  Ann N Y Acad Sci       Date:  1971-07-06       Impact factor: 5.691

6.  Correction of the enzymic defect in cultured fibroblasts from patients with Fabry's disease: treatment with purified alpha-galactosidase from ficin.

Authors:  G Dawson; R Matalon; Y T Li
Journal:  Pediatr Res       Date:  1973-08       Impact factor: 3.756

7.  The Hunter corrective factor. Purification and preliminary characterization.

Authors:  M Cantz; A Chrambach; G Bach; E F Neufeld
Journal:  J Biol Chem       Date:  1972-09-10       Impact factor: 5.157

8.  The defect in the Hurler and Scheie syndromes: deficiency of -L-iduronidase.

Authors:  G Bach; R Friedman; B Weissmann; E F Neufeld
Journal:  Proc Natl Acad Sci U S A       Date:  1972-08       Impact factor: 11.205

9.  The defect in the Hunter syndrome: deficiency of sulfoiduronate sulfatase.

Authors:  G Bach; F Eisenberg; M Cantz; E F Neufeld
Journal:  Proc Natl Acad Sci U S A       Date:  1973-07       Impact factor: 11.205

10.  Correction of abnormal cerebroside sulfate metabolism in cultured metachromatic leukodystrophy fibroblasts.

Authors:  M T Porter; A L Fluharty; H Kihara
Journal:  Science       Date:  1971-06-18       Impact factor: 47.728

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  4 in total

1.  Mucolipidosis type IV: abnormal transport of lipids to lysosomes.

Authors:  R Bargal; G Bach
Journal:  J Inherit Metab Dis       Date:  1997-09       Impact factor: 4.982

2.  Arylsulfatase A in pseudodeficiency.

Authors:  B Herz; G Bach
Journal:  Hum Genet       Date:  1984       Impact factor: 4.132

3.  A clinical trial of fibroblast transplantation for the treatment of mucopolysaccharidoses.

Authors:  D A Gibbs; E Spellacy; R Tompkins; R W Watts; J F Mowbray
Journal:  J Inherit Metab Dis       Date:  1983       Impact factor: 4.982

4.  Biochemical and histopathological studies on patients with mucopolysaccharidoses, two of whom had been treated by fibroblast transplantation.

Authors:  J Crow; D A Gibbs; W Cozens; E Spellacy; R W Watts
Journal:  J Clin Pathol       Date:  1983-04       Impact factor: 3.411

  4 in total

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