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


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… (More)


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