MSC p43 required for axonal development in motor neurons

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	MSC p43 required for axonal development in motor neurons
	Zhou, Jiawei
刊名	PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
	2009
卷号	106 期号:37 页码:15944-15949
关键词	assembly axon degeneration multisynthetase complex neurofilament TRANSFER-RNA SYNTHETASE MULTIFUNCTIONAL PROTEIN 1/P43 INTERMEDIATE-FILAMENTS TAIL DOMAINS DISEASE PHOSPHORYLATION ARCHITECTURE NEUROFILAMENTS TRANSPORT MUTATION
ISSN号	0027-8424
通讯作者	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
英文摘要	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.
学科主题	Science & Technology - Other Topics
收录类别	SCI
资助信息	Chinese Academy of Sciences; Chinese Ministry of Science and Technology[2006AA02Z184]; Shanghai Metropolitan Fund for Research and Development[07DJ14005]; Natural Science Foundation of China[30525041, 30623003, 30621130075]; State Key Program for Basic Research of China[2006CB500704]; Acceleration Research of KOSEF[370C-20090015]; 21st Frontier Functional Proteomics Research[370C-20090055]
语种	英语
公开日期	2012-07-13
内容类型	期刊论文
源URL	[http://ir.sibs.ac.cn/handle/331001/1643]
专题	上海神经科学研究所_神经所(总) 上海神经科学研究所_基底神经节的发育与退行性疾病研究组
推荐引用方式 GB/T 7714	Zhou, Jiawei. MSC p43 required for axonal development in motor neurons[J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA,2009,106(37):15944-15949.
APA	Zhou, Jiawei.(2009).MSC p43 required for axonal development in motor neurons.PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA,106(37),15944-15949.
MLA	Zhou, Jiawei."MSC p43 required for axonal development in motor neurons".PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA 106.37(2009):15944-15949.