Glycosylation and stem cells: Regulatory roles and application of iPSCs in the study of glycosylation-related disorders
Glycosylation and stem cells: Regulatory roles and application of iPSCs in the study of glycosylation-related disorders
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DOI：
10.1002/bies.201600138
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发表时间：
2016-12-01
期刊：
影响因子：
4
通讯作者：
Dalton, Stephen
中科院分区：
文献类型：
--
作者：
Berger, Ryan P.;Dookwah, Michelle;Dalton, Stephen
糖基化是指单糖或寡糖链对蛋白质和脂质的共翻译和翻译后修饰。哺乳动物细胞的表面被一系列异质且高度复杂的蛋白质和脂质连接的聚糖结构所装饰，这些结构在不同的细胞类型之间差异显着，这引发了关于它们在发育和疾病发病机制中的作用的问题。这篇综述将开始集中在最近的研究结果，确定在多能干细胞的细胞表面蛋白质和脂质糖基化的作用和它们的功能在正常发育过程中的影响。然后，我们将描述患者来源的诱导多能干细胞如何被用于模拟人类疾病，如先天性糖基化障碍。总的来说，这些研究表明，细胞表面聚糖在人类发育和疾病中起着关键作用。
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.