Influence of Sialic Acid on Cell Surface Properties in I-cell Disease Fibroblasts

Overview

Journal In Vitro

Specialties Anatomy & Histology
Cell Biology

Date 1981 Jul 1

PMID 7327591

Citations 1

Authors

G D Vladutiu

R M Fike

V T Amigone

Affiliations

Soon will be listed here.

Abstract

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.

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PMID: 6610362 PMC: 1900515.

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