Metabolism of mannose and fucose in cultured fibroblasts from patients with mannosidosis and mucolipidosis.
The incorporation of 14C-D-mannose or 14C-L-fucose into cultured fibroblasts from controls and patients with mannosidosis and mucolipidosis type II was studied. Mannose-containing oligosaccharide chains retarded on Sephadex G-25 were accumulated in cells from both patients. Fucose-containing oligosaccharides retarded on G-25 were also accumulated in cells from the patient with mucolipidosis. Besides there was an increase of macromolecular fucose-containing material in the cells from the patient with mucolipidosis. Conditioned medium of the cells from controls and the different patients exhibited about the same pattern as found in the cells, which might indicate leakage of the cellular material due to cell damage or death.
D002239:Carbohydrate Metabolism, Inborn Errors / Q000378:metabolism*; D002478:Cells, Cultured; D002850:Chromatography, Gel; D002854:Chromatography, Paper; D004586:Electrophoresis; D005347:Fibroblasts / Q000378:metabolism; D005643:Fucose / Q000378:metabolism*; D006801:Humans; D008358:Mannose / Q000378:metabolism*; D009081:Mucolipidoses / Q000378:metabolism*
D005643:Fucose; D008358:Mannose