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运动神经元存活蛋白在 mRNP 组装和运输中的作用。

A role for the survival of motor neuron protein in mRNP assembly and transport.

机构信息

Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, USA.

Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, USA; Department of Neurology, Emory University School of Medicine, Atlanta, GA, USA.

出版信息

Curr Opin Neurobiol. 2016 Aug;39:53-61. doi: 10.1016/j.conb.2016.04.004. Epub 2016 Apr 29.

Abstract

Localization and local translation of mRNA plays a key role in neuronal development and function. While studies in various systems have provided insights into molecular mechanisms of mRNA transport and local protein synthesis, the factors that control the assembly of mRNAs and mRNA binding proteins into messenger ribonucleoprotein (mRNP) transport granules remain largely unknown. In this review we will discuss how insights on a motor neuron disease, spinal muscular atrophy (SMA), is advancing our understanding of regulated assembly of transport competent mRNPs and how defects in their assembly and delivery may contribute to the degeneration of motor neurons observed in SMA and other neurological disorders.

摘要

mRNA 的定位和局部翻译在神经元发育和功能中起着关键作用。虽然在不同系统中的研究提供了有关 mRNA 运输和局部蛋白质合成的分子机制的见解,但控制 mRNA 和 mRNA 结合蛋白组装成信使核糖核蛋白(mRNP)运输颗粒的因素在很大程度上仍然未知。在这篇综述中,我们将讨论运动神经元疾病脊髓性肌萎缩症(SMA)的研究如何增进我们对运输能力 mRNP 调节组装的理解,以及它们的组装和运输缺陷如何导致 SMA 和其他神经疾病中观察到的运动神经元退化。

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