Biological role of dystroglycan in Schwann cell function and its implications in peripheral nervous system diseases.

Biological role of dystroglycan in Schwann cell function and its implications in peripheral nervous system diseases.
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DOI:
10.1155/2010/740403
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发表时间:
2010
影响因子:
--
通讯作者:
Matsumura K
Matsumura K
中科院分区:
其他
文献类型:
--
作者:
Masaki T;Matsumura K

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抗肌营养不良多糖是抗肌营养不良蛋白-糖蛋白复合体(DGC)的中心成分,它连接细胞外基质和细胞骨架,在各种胎儿和成人组织中都有表达。营养不良多糖在发育和动态平衡中发挥着多种作用,包括基底膜的形成、上皮的形态发生、膜的稳定性、细胞的极化和细胞的迁移。在本文中,我们将重点介绍营养不良多糖在雪旺细胞功能,特别是髓鞘形成中的生物学作用。首先,我们回顾了雪旺细胞无轴突膜上DGC的分子结构。然后,我们将回顾利用定向突变进行的功能丧失研究,这些研究揭示了雪旺细胞中DGC的每一种成分的生物学功能。基于这些发现,将详细讨论营养不良多糖在雪旺细胞功能中的作用,特别是在髓鞘形成中的作用,以及它在疾病中的意义。最后,鉴于了解营养不良多糖在雪旺细胞中的作用才刚刚开始,未来的前景将被讨论。
Dystroglycan is a central component of the dystrophin-glycoprotein complex (DGC) that links extracellular matrix with cytoskeleton, expressed in a variety of fetal and adult tissues. Dystroglycan plays diverse roles in development and homeostasis including basement membrane formation, epithelial morphogenesis, membrane stability, cell polarization, and cell migration. In this paper, we will focus on biological role of dystroglycan in Schwann cell function, especially myelination. First, we review the molecular architecture of DGC in Schwann cell abaxonal membrane. Then, we will review the loss-of-function studies using targeted mutagenesis, which have revealed biological functions of each component of DGC in Schwann cells. Based on these findings, roles of dystroglycan in Schwann cell function, in myelination in particular, and its implications in diseases will be discussed in detail. Finally, in view of the fact that understanding the role of dystroglycan in Schwann cells is just beginning, future perspectives will be discussed.
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