| Carbohydrate-deficient glycoprotein syndrome: not an N-linked oligosaccharide processing defect, but an abnormality in lipid-linked oligosaccharide biosynthesis? | |
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MedLine Citation:
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PMID: 7962535 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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L D Powell; K Paneerselvam; R Vij; S Diaz; A Manzi; N Buist; H Freeze; A Varki |
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Publication Detail:
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Type: Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: The Journal of clinical investigation Volume: 94 ISSN: 0021-9738 ISO Abbreviation: J. Clin. Invest. Publication Date: 1994 Nov |
Date Detail:
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Created Date: 1994-12-02 Completed Date: 1994-12-02 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 7802877 Medline TA: J Clin Invest Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1901-9 Citation Subset: AIM; IM |
Affiliation:
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Glycobiology Program, University of California at San Diego Cancer Center, La Jolla 92093. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Blood Proteins
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analysis Carbohydrates / analysis* Cell Line Female Glycoproteins / analysis, blood* Glycosylation Humans Isoelectric Focusing Lipid Metabolism Mannose / metabolism Oligosaccharides / biosynthesis* Syndrome Transferrin / chemistry* |
| Grant Support | |
ID/Acronym/Agency:
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KO8 CA01649/CA/NCI NIH HHS; R01 GM49096/GM/NIGMS NIH HHS; R01-CA38701/CA/NCI NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Blood Proteins; 0/Carbohydrates; 0/Glycoproteins; 0/Oligosaccharides; 11096-37-0/Transferrin; 31103-86-3/Mannose |
| Comments/Corrections | |
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