Program of Physiology and Biophysics, Institute of Biomedical Sciences (ICBM) and Biomedical Neuroscience Institute (BNI), Faculty of Medicine, Universidad de Chile, Santiago, Chile.
Program of Physiology and Biophysics, Institute of Biomedical Sciences (ICBM) and Biomedical Neuroscience Institute (BNI), Faculty of Medicine, Universidad de Chile, Santiago, Chile.
Semin Cell Dev Biol. 2014 Mar;27:23-31. doi: 10.1016/j.semcdb.2013.12.004. Epub 2013 Dec 19.
Neurons are responsible for the generation and propagation of electrical impulses, which constitute the central mechanism of information transfer between the nervous system and internal or external environments. Neurons are large and polarized cells with dendrites and axons constituting their major functional domains. Axons are thin and extremely long specializations that mediate the conduction of these electrical impulses. Regulation of the axonal proteome is fundamental to generate and maintain neural function. Although classical mechanisms of protein transport have been around for decades, a variety newly identified mechanisms to control the abundance of axonal proteins have appeared in recent years. Here we briefly describe the classical models of axonal transport and compare them to the emerging concepts of axonal biosynthesis centered on the endoplasmic reticulum. We review the structure of the axonal endoplasmic reticulum, and its role in diffusion and trafficking of axonal proteins. We also analyze the contribution of other secretory organelles to axonal trafficking and evaluate the potential consequences of axonal endoplasmic reticulum malfunction in neuropathology.
神经元负责产生和传播电脉冲,这构成了神经系统与内部或外部环境之间信息传递的核心机制。神经元是大型的极化细胞,其树突和轴突构成其主要的功能域。轴突是细长的特殊结构,介导这些电脉冲的传导。轴突蛋白质组的调节对于产生和维持神经功能至关重要。尽管经典的蛋白质运输机制已经存在了几十年,但近年来出现了多种新的控制轴突蛋白丰度的机制。在这里,我们简要描述了经典的轴突运输模型,并将其与以内质网为中心的新兴轴突生物合成概念进行了比较。我们回顾了轴突内质网的结构及其在轴突蛋白扩散和运输中的作用。我们还分析了其他分泌细胞器对轴突运输的贡献,并评估了轴突内质网功能障碍在神经病理学中的潜在后果。