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在体内表达的 Arf6 GEF cytohesin-1 在小鼠中表现出增强的神经髓鞘厚度。

In vivo expression of the Arf6 Guanine-nucleotide exchange factor cytohesin-1 in mice exhibits enhanced myelin thickness in nerves.

机构信息

Molecular Pharmacology Group, Department of Pharmacology, National Research Institute for Child Health and Development, 2-10-1 Okura, Setagaya, Tokyo, 157-8535, Japan.

出版信息

J Mol Neurosci. 2013 Oct;51(2):522-31. doi: 10.1007/s12031-013-0018-4. Epub 2013 May 1.

Abstract

The myelin sheath consists of a unique multiple layer structure that acts as an insulator between neuronal axons to enhance the propagation of the action potential. In neuropathies such as demyelinating or dismyelinating diseases, chronic demyelination and defective remyelination occur repeatedly, leading to more severe neuropathy. As yet, little is known about the possibility of drug target-specific medicine for such diseases. In the developing peripheral nervous system (PNS), myelin sheaths form as Schwann cells wrap individual axons. It is thought that the development of a drug promoting myelination by Schwann cells would provide effective therapy against peripheral nerve disorders: to test such treatment, genetically modified mice overexpressing the drug target molecules are needed. We previously identified an Arf6 activator, the guanine-nucleotide exchange factor cytohesin-1, as the signaling molecule controlling myelination of peripheral axons by Schwann cells; yet, the important issue of whether cytohesin-1 itself promotes myelin thickness in vivo has remained unclear. Herein, we show that, in mouse PNS nerves, Schwann cell-specific expression of wild-type cytohesin-1 exhibits enhanced myelin thickness. Downstream activation of Arf6 is also seen in these transgenic mice, revealing the involvement of the cytohesin-1 and Arf6 signaling unit in promoting myelination. These results suggest that cytohesin-1 may be a candidate for the basis of a therapy for peripheral neuropathies through its enhancement of myelin thickness.

摘要

髓鞘由独特的多层结构组成,作为神经元轴突之间的绝缘体,以增强动作电位的传播。在脱髓鞘或脱髓鞘疾病等神经病变中,慢性脱髓鞘和有缺陷的髓鞘再生反复发生,导致更严重的神经病变。到目前为止,对于这种疾病,药物靶点特异性药物的可能性知之甚少。在发育中的周围神经系统 (PNS) 中,施万细胞包裹单个轴突形成髓鞘。人们认为,促进施万细胞髓鞘形成的药物的开发将为周围神经紊乱提供有效的治疗方法:为了测试这种治疗方法,需要过表达药物靶点分子的基因修饰小鼠。我们之前确定了一种 Arf6 激活剂,即鸟嘌呤核苷酸交换因子细胞松弛素-1,作为控制施万细胞对周围轴突髓鞘形成的信号分子;然而,细胞松弛素-1 本身是否在体内促进髓鞘厚度的重要问题仍不清楚。在此,我们表明在小鼠 PNS 神经中,野生型细胞松弛素-1的施万细胞特异性表达表现出增强的髓鞘厚度。这些转基因小鼠中也观察到 Arf6 的下游激活,表明细胞松弛素-1 和 Arf6 信号单元参与促进髓鞘形成。这些结果表明,细胞松弛素-1 可能通过增强髓鞘厚度成为治疗周围神经病变的候选药物。

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