Körner, Christian and Lehle, Ludwig and von Figura, Kurt (1998) Carbohydrate-deficient glycoprotein syndrome type 1: correction of the glycosylation defect by deprivation of glucose or supplementation of mannose. GLYCOCONJUGATE JOURNAL, 15 (5). pp. 499-505. ISSN 0282-0080,
Full text not available from this repository.Abstract
In the carbohydrate deficient glycoprotein syndrome (CDGS) type 1 glycoproteins with less and shorter N-linked oligosaccharides are synthesized due to a deficiency of phosphomannomutase. Glucose deprivation or mannose addition are shown to partially or fully correct the size of oligosaccharides incorporated into lipid linked oligosaccharides and nascent glycoproteins in skin fibroblasts from CDGS type 1 patients with a phosphomannomutase defect, The corrective effect is ascribed to regulatory mechanisms and/or metabolic pathways that bypass phosphomannomutase.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | LINKED OLIGOSACCHARIDE BIOSYNTHESIS; SYNDROME TYPE-I; MAMMALIAN-CELLS; STARVATION; CDGS type 1; mannose addition; glucose deprivation |
| Subjects: | 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: | 23 May 2023 12:59 |
| Last Modified: | 23 May 2023 12:59 |
| URI: | https://pred.uni-regensburg.de/id/eprint/49837 |
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