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Title: MSC p43 required for axonal development in motor neurons
Author: Zhu, Xiaodong ; Liu, Yang ; Yin, Yanqing ; Shao, Aiyun ; Zhang, Bo ; Kim, Sunghoon ; null(周嘉伟)
Source: PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
Issued Date: 2009
Volume: 106, Issue:37, Pages:15944-15949
Keyword: assembly ; axon degeneration ; multisynthetase complex ; neurofilament ; TRANSFER-RNA SYNTHETASE ; MULTIFUNCTIONAL PROTEIN 1/P43 ; INTERMEDIATE-FILAMENTS ; TAIL DOMAINS ; DISEASE ; PHOSPHORYLATION ; ARCHITECTURE ; NEUROFILAMENTS ; TRANSPORT ; MUTATION
Subject: Science & Technology - Other Topics
Corresponding Author: Zhou, JW (reprint author), Chinese Acad Sci, Shanghai Inst Biol Sci, State Key Lab Neurosci, Inst Neurosci, Shanghai 200031, Peoples R China,jwzhou@ion.ac.cn
English Abstract: Neuron connectivity and correct neural function largely depend on axonal integrity. Neurofilaments (NFs) constitute the main cytoskeletal 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 phosphorylation 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.
Indexed Type: sci
Language: 英语
Content Type: 期刊论文
URI: http://ir.sibs.ac.cn/handle/331001/1643
Appears in Collections:神经所(总)_期刊论文
基底神经节的发育与退行性疾病研究组_期刊论文

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Zhu, Xiaodong; Liu, Yang; Yin, Yanqing; Shao, Aiyun; Zhang, Bo; Kim, Sunghoon; Zhou, Jiawei.MSC p43 required for axonal development in motor neurons,PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA,2009,106(37):15944-15949
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