Abstract
Extract: Cultured skin fibroblasts from normal individuals and patients with Hurler's syndrome were examined by electron microscopy at different phases of the culture cycle at 7, 21, and 35 days after retrypsinization and inoculation. Cultures were incubated for 0.5–24 hr with colloidal gold, incubated to localize acid phosphatase and aryl sulfatase B activities histochemically, and assayed for these enzymes biochemically. Both types of fibroblasts grew to saturation densities of 4 × 106 cells/dish.
The large vacuoles seen in the cytoplasm of Hurler fibroblasts appeared to be secondary lysosomes, for they accumulated particles of colloidal gold and contained myelin-like figures. They seemed to derive from the Golgi membranes which tended to show a higher frequency of dilated cisternae than those of normal cells. Specific activities of the assayed enzymes in normal and Hurler cells were equal. In normal cells, acid phosphatase was demonstrated in the same vacuoles which accumulated colloidal gold.
Speculation: The large vacuoles of Hurler fibroblasts appear to be secondary lysosomes and to derive from the Golgi apparatus rather than from the rough endoplasmic reticulum.
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Conrad, G., Sherman, D. & Dorfman, A. An Ultrastructural Comparison of Normal and Hurler Syndrome Dermal Fibroblasts. Pediatr Res 6, 563–575 (1972). https://doi.org/10.1203/00006450-197206000-00005
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DOI: https://doi.org/10.1203/00006450-197206000-00005
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