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The correction of hunter fibroblasts by exogenous iduronate sulfate sulfatase: Biochemical and ultrastructural studies

Authors
  • Rachel Eliahu
  • Eliezer Sekeles
  • Rachel Cohen
  • Gideon Bach
Publication Date
Jul 01, 1981
Source
PMC
Keywords
Disciplines
  • Biology
  • Chemistry
License
Unknown

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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