Glycosylation and stem cells: Regulatory roles and application of iPSCs in the study of glycosylation-related disorders

Glycosylation refers to the co‐ and post‐translational modification of protein and lipids by monosaccharides or oligosaccharide chains. The surface of mammalian cells is decorated by a heterogeneous and highly complex array of protein and lipid linked glycan structures that vary significantly betwee...

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Bibliographic Details
Published in:BioEssays 2016-12, Vol.38 (12), p.1255-1265
Main Authors: Berger, Ryan P., Dookwah, Michelle, Steet, Richard, Dalton, Stephen
Format: Article
Language:English
Subjects:
Animals
congenital abnormalities
congenital disorders of glycosylation
Glycosylation
human development
human diseases
Humans
induced pluripotent stem cells
Induced Pluripotent Stem Cells - metabolism
lipids
Lipids - chemistry
mammals
Metabolism, Inborn Errors - metabolism
monosaccharides
oligosaccharides
pathogenesis
patients
pluripotent stem cells
polysaccharides
Proteins
Proteins - chemistry
Proteins - metabolism
Stem cells
surface proteins
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Summary:Glycosylation refers to the co‐ and post‐translational modification of protein and lipids by monosaccharides or oligosaccharide chains. The surface of mammalian cells is decorated by a heterogeneous and highly complex array of protein and lipid linked glycan structures that vary significantly between different cell types, raising questions about their roles in development and disease pathogenesis. This review will begin by focusing on recent findings that define roles for cell surface protein and lipid glycosylation in pluripotent stem cells and their functional impact during normal development. Then, we will describe how patient derived induced pluripotent stem cells are being used to model human diseases such as congenital disorders of glycosylation. Collectively, these studies indicate that cell surface glycans perform critical roles in human development and disease. Glycan structures serve as signatures for stem cell identity and play important roles in regulating differentiation toward specific lineages. The use of induced pluripotent stem cells (iPSCs) and iPSC‐derived cell lines from patients with glycosylation disorders provide valuable new cell models to study disease pathogenesis and test potential therapies.
ISSN:0265-9247
1521-1878
DOI:10.1002/bies.201600138