Abstract | BACKGROUND: SCOPE OF REVIEW: MAJOR CONCLUSIONS: Both UDP-GlcNAc and CMP-sialic acid are important precursors for diverse protein glycosylation reactions and for conversion into other nucleotide- sugars. Defects in the synthesis of these nucleotide sugars might affect a wide range of protein glycosylation reactions. Involvement of multiple glycosylation pathways might contribute to disease phenotype, but the currently available biochemical information on sugar metabolism is insufficient to understand why defects in these pathways present with tissue-specific phenotypes. GENERAL SIGNIFICANCE: Future research on the interplay between sugar metabolism and different glycosylation pathways in a tissue- and cell-specific manner will contribute to elucidation of disease mechanisms and will create new opportunities for therapeutic intervention. This article is part of a Special Issue entitled " Glycans in personalised medicine" Guest Editor: Professor Gordan Lauc.
|
Authors | Anke P Willems, Baziel G M van Engelen, Dirk J Lefeber |
Journal | Biochimica et biophysica acta
(Biochim Biophys Acta)
Vol. 1860
Issue 8
Pg. 1640-54
(Aug 2016)
ISSN: 0006-3002 [Print] Netherlands |
PMID | 26721333
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
|
Copyright | Copyright © 2016 Elsevier B.V. All rights reserved. |
Chemical References |
- Glycoproteins
- Hexosamines
- GFPT1 protein, human
- Glutamine-Fructose-6-Phosphate Transaminase (Isomerizing)
- PGM3 protein, human
- Phosphoglucomutase
- N-Acetylneuraminic Acid
|
Topics |
- Adult
- Carbohydrate Metabolism, Inborn Errors
(genetics, metabolism)
- Glutamine-Fructose-6-Phosphate Transaminase (Isomerizing)
(genetics, metabolism)
- Glycoproteins
(genetics, metabolism)
- Glycosylation
- Hexosamines
(genetics, metabolism)
- Humans
- N-Acetylneuraminic Acid
(genetics, metabolism)
- Phosphoglucomutase
(genetics, metabolism)
|