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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >MSC p43 required for axonal development in motor neurons
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MSC p43 required for axonal development in motor neurons

机译：运动神经元轴突发育所需的MSC p43

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摘要

Neuron connectivity and correct neural function largely depend on axonal integrity. Neurofilaments (NFs) constitute the main cy-toskeletal network maintaining the structural integrity of neurons and exhibit dynamic changes during axonal and dendritic growth. However, the mechanisms underlying axonal development and maintenance remain poorly understood. Here, we identify that multisynthetase complex p43 (MSC p43) is essential for NF assembly and axon maintenance. The MSC p43 protein was predominantly expressed in central neurons and interacted with NF light subunit in vivo. Mice lacking MSC p43 exhibited axon degeneration in motor neurons, defective neuromuscular junctions, muscular atrophy, and motor dysfunction. Furthermore, MSC p43 depletion in mice caused disorganization of the axonal NF network. Mechanistically, MSC p43 is required for maintaining normal phosphor-ylation levels of NFs. Thus, MSC p43 is indispensable in maintaining axonal integrity. Its dysfunction may underlie the NF disorganization and axon degeneration associated with motor neuron degenerative diseases.
机译：神经元的连通性和正确的神经功能在很大程度上取决于轴突的完整性。神经丝（NFs）构成主要的cy-骨骼网络，维持神经元的结构完整性，并在轴突和树突状生长过程中表现出动态变化。然而，对轴突发育和维持的机制仍知之甚少。在这里，我们确定多合成酶复合物p43（MSC p43）对于NF组装和轴突维护至关重要。 MSC p43蛋白主要在中枢神经元中表达，并在体内与NF light亚基相互作用。缺乏MSC p43的小鼠在运动神经元，神经肌肉接头缺陷，肌肉萎缩和运动功能障碍方面表现出轴突变性。此外，小鼠中MSC p43的耗竭导致轴突NF网络的混乱。从机理上讲，需要MSC p43来维持NF的正常磷酸化水平。因此，MSC p43是维持轴突完整性必不可少的。它的功能障碍可能是与运动神经元变性疾病相关的NF紊乱和轴突变性的基础。

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  • 作者

    Xiaodong Zhu; Yang Liu; Yanqing Yin; Aiyun Shao; Bo Zhang; Sunghoon Kim; Jiawei Zhou;

  • 作者单位

    Laboratory of Molecular Cell Biology, Institute of Biochemistry and Cell Biology, Chinese Academy of Sciences, Shanghai 200031, China State Key Laboratory of Neuroscience Institute of Neuroscience, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China;

    State Key Laboratory of Neuroscience Institute of Neuroscience, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China;

    State Key Laboratory of Neuroscience Institute of Neuroscience, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China;

    State Key Laboratory of Neuroscience Institute of Neuroscience, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China;

    State Key Laboratory of Neuroscience Institute of Neuroscience, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China;

    Center for Medicinal Protein Network and Systems Biology, College of Pharmacy, Seoul National University, Seoul 151-742, Korea;

    State Key Laboratory of Neuroscience Institute of Neuroscience, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China;

  • 收录信息 美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类
  • 关键词

    assembly; axon degeneration; multisynthetase complex; neurofilament;

    机译：部件;轴突变性多合成酶复合物;神经丝;

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