Enzyme replacement in Fabry endothelial cells and fibroblasts: uptake experiments and electron microscopical studies.
作者： L. Hasholt , A. Wandall , S. A. Sørensen
DOI: 10.1111/J.1399-0004.1988.TB03463.X
关键词:
摘要: Endothelial cells are of particular interest for therapeutic strategies in Fabry's disease, because the accumulation glycosphingolipids vascular endothelium as a result alpha-galactosidase A (alpha-galA) deficiency is responsible major clinical manifestations disease. Electron microscopical observations cultured endothelial obtained from umbilical vein hemizygous Fabry fetus showed that deposited lamellar material lysosomes, has been found previously fibroblasts and many different tissues. Mannose 6-phosphate (man 6-P)-receptor mediated Concanavalin (ConA)-mediated uptake purified alpha-galA was attempted well same fetus. Our results on high-uptake indicate do not internalize via man 6-P receptor. Immunofluorescence studies after addition receptor antibody to support theory they have no or very few receptors surface. Morphological did show lysosomal changes which could suggest enzyme taken up into cells; however, we reproducible modifications lysosomes incubation with alpha-galA. Cell-associated activity both cell types, when added preincubated ConA; but lectin treatment by itself induced considerable ultrastructural cytoplasm, obscured possible effect enzyme.
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