dolichol-monophosphate-mannose has been researched along with mannose-1-phosphate* in 1 studies
1 other study(ies) available for dolichol-monophosphate-mannose and mannose-1-phosphate
Article | Year |
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Abnormal synthesis of mannose 1-phosphate derived carbohydrates in carbohydrate-deficient glycoprotein syndrome type I fibroblasts with phosphomannomutase deficiency.
In fibroblasts from five patients with carbohydrate-deficient glycoprotein syndrome type 1, the incorporation of [2-3H] mannose into mannose phosphates, GDP-mannose, GDP-fucose, dolichol-P-mannose, lipid-linked oligosaccharides, and glycoprotein fraction was determined. We observed a 3- to 5-fold reduction of incorporation of radioactivity into mannose 1-phosphate, GDP-mannose, GDP-fucose, dolichol-P-mannose, and nascent glycoproteins. The incorporation of radioactivity into mannose 6-phosphate was normal. The formation of lipid linked oligosaccharides was only slightly affected (=20%), but their size was severely reduced, mostly containing five or fewer residues. As a consequence, truncated oligosaccharides were transferred to newly synthesized glycoproteins. The metabolic changes can be explained by a deficiency of phosphomannomutase activity, which was reduced to =10% of control. Topics: Carbohydrates; Cells, Cultured; Congenital Disorders of Glycosylation; Dolichol Monophosphate Mannose; Fibroblasts; Guanosine Diphosphate Fucose; Guanosine Diphosphate Mannose; Humans; Lipopolysaccharides; Mannosephosphates; Phosphotransferases (Phosphomutases) | 1998 |