| Autor: |
Vladutiu GD, Fike RM, Amigone VT |
| Jazyk: |
angličtina |
| Zdroj: |
In vitro [In Vitro] 1981 Jul; Vol. 17 (7), pp. 588-92. |
| DOI: |
10.1007/BF02618456 |
| Abstrakt: |
Fibroblasts derived from patients with I-cell disease have been shown to accumulate many natural substrates including a three to fourfold increase in sialic acid content compared to that found in normal fibroblasts. This diverse accumulation of storage material is due to a massive deficiency of multiple lysosomal hydrolases as they are preferentially excreted into the culture fluid. There is evidence that the I-cell plasma membrane itself is abnormal with respect to certain transferase activities and in its sensitivity to freezing and Triton X-100. In this study, we have shown that a neuraminidase-sensitive substrate, and perhaps others in I-cell fibroblasts, contribute to an increased electronegativity if the I-cell fibroblast surface and to the cells' sensitivity to freezing. We also found that neuraminidase treatment of I-cell fibroblasts before preservative freezing in liquid nitrogen enables the cells to adapt more easily to subculture upon thawing. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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