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层粘连蛋白在周围神经发育和肌肉营养不良中的作用

Laminins in peripheral nerve development and muscular dystrophy.

作者信息

Yu Wei-Ming, Yu Huaxu, Chen Zu-Lin

机构信息

Laboratory of Neurobiology and Genetics, The Rockefeller University, 1230 York Avenue, New York, NY, 10021, USA.

出版信息

Mol Neurobiol. 2007 Jun;35(3):288-97. doi: 10.1007/s12035-007-0026-x.

Abstract

Laminins are extracellular matrix (ECM) proteins that play an important role in cellular function and tissue morphogenesis. In the peripheral nervous system (PNS), laminins are expressed in Schwann cells and participate in their development. Mutations in laminin subunits expressed in the PNS and in skeleton muscle may cause peripheral neuropathies and muscular dystrophy in both humans and mice. Recent studies using gene knockout technology, such as cell-type specific gene targeting techniques, revealed that laminins and their receptors mediate Schwann cell and axon interactions. Schwann cells with disrupted laminin expression exhibit impaired proliferation and differentiation and also undergo apoptosis. In this review, we focus on the potential molecular mechanisms by which laminins participate in the development of Schwann cells.

摘要

层粘连蛋白是细胞外基质(ECM)蛋白,在细胞功能和组织形态发生中发挥重要作用。在周围神经系统(PNS)中,层粘连蛋白在施万细胞中表达并参与其发育。在PNS和骨骼肌中表达的层粘连蛋白亚基的突变可能导致人类和小鼠的周围神经病变和肌肉萎缩症。最近使用基因敲除技术(如细胞类型特异性基因靶向技术)的研究表明,层粘连蛋白及其受体介导施万细胞和轴突的相互作用。层粘连蛋白表达受损的施万细胞表现出增殖和分化受损,并且还会发生凋亡。在这篇综述中,我们重点关注层粘连蛋白参与施万细胞发育的潜在分子机制。

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