Körner, Christian and Lehle, Ludwig and von Figura, Kurt (1998) Abnormal synthesis of mannose 1-phosphate derived carbohydrates in carbohydrate-deficient glycoprotein syndrome type I fibroblasts with phosphomannomutase deficiency. GLYCOBIOLOGY, 8 (2). pp. 165-171. ISSN 0959-6658, 1460-2423
Full text not available from this repository.Abstract
In fibroblasts from five patients with carbohydrate-deficient glycoprotein syndrome type 1, the incorporation of [2-H-3] 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 l-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 (less than or equal to 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 less than or equal to 10% of control.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | SERUM TRANSFERRIN; OLIGOSACCHARIDES; DOLICHOL; BIOSYNTHESIS; ADHESION; DEFECT; GOLGI; LEWIS; phosphomannomutase deficiency; CDGS type I; mannose metabolites |
| Subjects: | 500 Science > 570 Life sciences 500 Science > 580 Botanical sciences |
| Divisions: | Biology, Preclinical Medicine > Institut für Pflanzenwissenschaften > Lehrstuhl für Zellbiologie und Pflanzenphysiologie (Prof. Dr. Klaus Grasser) |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 28 Mar 2023 04:39 |
| Last Modified: | 28 Mar 2023 04:39 |
| URI: | https://pred.uni-regensburg.de/id/eprint/50101 |
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