Influence of sialic acid on cell surface properties in I-cell disease fibroblasts |
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Authors: | Georgirene D Vladutiu Richard M Fike Valerie T Amigone |
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Institution: | (1) Department of Pediatrics at Children's Hospital, State University of New York at Buffalo, 14222 Buffalo, New York |
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Abstract: | Summary 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 of 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.
This project was supported in part by National Institutes of Health (NIH) BRSG Grant RR-05493, NIH Grant 1-R01-HD-11453-01-A1,
National Science Foundation Grant PCM 77-05733, and Maternal and Child Health Service Project 417. Georgirene D. Vladutiu
is the recipient of Research Career Development Award 1K04 HD 00312-01A1 from the National Institutes of Health. |
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Keywords: | sialic acid I-cell disease cell electrophoresis cryopreservation neuraminidase |
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