ArticleGlycoconjugate journal1998
Carbohydrate-deficient glycoprotein syndrome type 1: correction of the glycosylation defect by deprivation of glucose or supplementation of mannose.
Article in Glycoconjugate journal, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
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Who cites it
15 citing papers in PubMed, 41 citations in OpenAlex.
- Liposome-encapsulated mannose-1-phosphate therapy improves global N-glycosylation in different congenital disorders of glycosylation.Molecular genetics and metabolism · 2024Article
- Dietary mannose supplementation in phosphomannomutase 2 deficiency (PMM2-CDG).Orphanet journal of rare diseases · 2020Article
- Unsuccessful intravenous D-mannose treatment in PMM2-CDG.Orphanet journal of rare diseases · 2019Article
- CDG Therapies: From Bench to Bedside.International journal of molecular sciences · 2018Review
- Glycomic Characterization of Induced Pluripotent Stem Cells Derived from a Patient Suffering from Phosphomannomutase 2 Congenital Disorder of Glycosylation (PMM2-CDG).Molecular & cellular proteomics : MCP · 2016Article
- Therapies and therapeutic approaches in Congenital Disorders of Glycosylation.Glycoconjugate journal · 2013Review
- Successful prenatal mannose treatment for congenital disorder of glycosylation-Ia in mice.Nature medicine · 2011Article
- High throughput isolation and glycosylation analysis of IgG-variability and heritability of the IgG glycome in three isolated human populations.Molecular & cellular proteomics : MCP · 2011Article
- Towards a therapy for phosphomannomutase 2 deficiency, the defect in CDG-Ia patients.Biochimica et biophysica acta · 2009Review
- Targeted disruption of the mouse phosphomannomutase 2 gene causes early embryonic lethality.Molecular and cellular biology · 2006Article
- Analysis of glycosylation in CDG-Ia fibroblasts by fluorophore-assisted carbohydrate electrophoresis: implications for extracellular glucose and intracellular mannose 6-phosphate.The Journal of biological chemistry · 2005Article
- Congenital disorders of glycosylation: review of their molecular bases, clinical presentations and specific therapies.European journal of pediatrics · 2003Review
- Article
- Deficiency of dolichol-phosphate-mannose synthase-1 causes congenital disorder of glycosylation type Ie.The Journal of clinical investigation · 2000Article
- Regulation of the dolichol pathway in human fibroblasts by the endoplasmic reticulum unfolded protein response.Proceedings of the National Academy of Sciences of the United States of America · 1999Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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.
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Registered trials
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