ArticleThe Journal of clinical investigation1994
Carbohydrate-deficient glycoprotein syndrome: not an N-linked oligosaccharide processing defect, but an abnormality in lipid-linked oligosaccharide biosynthesis?
Article in The Journal of clinical investigation, 1994. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 71 citations in OpenAlex.
- Genotypes and phenotypes of patients in the UK with carbohydrate-deficient glycoprotein syndrome type 1.Journal of inherited metabolic disease · 2000Trial
- Biological roles of glycans.Glycobiology · 2017Review
- Lipopolysaccharide induced upregulation of beta-1,4-galactosyltransferase-I in Schwann cell.Inflammation · 2009Article
- 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
- Involvement of beta 1,4 galactosyltransferase 1 and Gal beta1-->4GlcNAc groups in human hepatocarcinoma cell apoptosis.Molecular and cellular biochemistry · 2003Article
- 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
- The sugar moiety of Tamm-Horsfall protein is affected by the carbohydrate-deficient glycoprotein type I syndrome. A case study.Glycoconjugate journal · 1999Article
- Kinetic properties and tissular distribution of mammalian phosphomannomutase isozymes.The Biochemical journal · 1999Article
- A novel carbohydrate-deficient glycoprotein syndrome characterized by a deficiency in glucosylation of the dolichol-linked oligosaccharide.The Journal of clinical investigation · 1998Article
- Carbohydrate-deficient glycoprotein syndrome type 1: correction of the glycosylation defect by deprivation of glucose or supplementation of mannose.Glycoconjugate journal · 1998Article
- Carbohydrate-deficient glycoprotein syndrome type Ib. Phosphomannose isomerase deficiency and mannose therapy.The Journal of clinical investigation · 1998Article
- Carbohydrate deficient glycoprotein (CDG) syndrome type I.Journal of medical genetics · 1997Article
- The identification of abnormal glycoforms of serum transferrin in carbohydrate deficient glycoprotein syndrome type I by capillary zone electrophoresis.Glycoconjugate journal · 1996Article
- Mutations in the MGAT2 gene controlling complex N-glycan synthesis cause carbohydrate-deficient glycoprotein syndrome type II, an autosomal recessive disease with defective brain development.American journal of human genetics · 1996Article
- Mannose corrects altered N-glycosylation in carbohydrate-deficient glycoprotein syndrome fibroblasts.The Journal of clinical investigation · 1996Article
- Carbohydrate-deficient glycoprotein syndrome type I: determination of the oligosaccharide structure of newly synthesized glycoproteins by analysis of calnexin binding.Journal of inherited metabolic disease · 1996Article
- Carbohydrate-deficient transferrin values in neonatal and umbilical cord blood.Journal of inherited metabolic disease · 1996Article
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
Abstract
The carbohydrate-deficient glycoprotein syndrome (CDGS) is a developmental disease associated with an abnormally high isoelectric point of serum transferrin. Carbohydrate analyses of this glycoprotein initially suggested a defect in N-linked oligosaccharide processing, although more recent studies indicate a defect in the attachment of these sugar chains to the protein. We studied both serum glycoproteins and fibroblast-derived [2-3H]mannose-labeled oligosaccharides from CDGS patients and normal controls. While there was a decrease in the glycosylation of serum glycoproteins of affected individuals, differences were not seen in either monosaccharide composition or oligosaccharide structures. The lectin-binding profiles of glycopeptides from [2-3H]-mannose-labeled fibroblasts were likewise indistinguishable. However, the incorporation of [2-3H]mannose into both glycoproteins and the dolichol-linked oligosaccharide precursor was significantly reduced. Thus, at least in some patients, CDGS is not due to a defect in processing of N-linked oligosaccharides, but rather to defective synthesis and transfer of nascent dolichol-linked oligosaccharide precursors. This abnormality could result in both a failure to glycosylate some sites on some proteins, as well as secondary abnormalities in overall glycoprotein processing and/or function.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.