Viral Transduction of trkA into Cultured Nodose and Spinal Motor Neurons Conveys NGF Responsiveness

1994 ◽  
Vol 163 (1) ◽  
pp. 152-161 ◽  
Author(s):  
Hong Xu ◽  
Howard Federoff ◽  
Johnna Maragos ◽  
Luis F. Parada ◽  
John A. Kessler
Keyword(s):  
Motor Neurons ◽  
Spinal Motor Neurons ◽  
Spinal Motor ◽  
Viral Transduction

scholarly journals Translatomic analysis of regenerating and degenerating spinal motor neurons in injury and ALS

iScience ◽  
2021 ◽  
pp. 102700
Author(s):  
Jennifer L. Shadrach ◽  
Wesley M. Stansberry ◽  
Allison M. Milen ◽  
Rachel E. Ives ◽  
Elizabeth A. Fogarty ◽  
...  
Keyword(s):  
Motor Neurons ◽  
Spinal Motor Neurons ◽  
Spinal Motor

A new method of isolating spinal motor neurons from fetal mouse

2017 ◽  
Vol 288 ◽  
pp. 57-61 ◽  
Author(s):  
Weifang Wang ◽  
Bao Qi ◽  
Hui Lv ◽  
Fei Wu ◽  
Lulu Liu ◽  
...  
Keyword(s):  
Motor Neurons ◽  
New Method ◽  
Fetal Mouse ◽  
Spinal Motor Neurons ◽  
Spinal Motor

scholarly journals Genetic ablation of dynactin p150Glued in postnatal neurons causes preferential degeneration of spinal motor neurons in aged mice

2018 ◽  
Vol 13 (1) ◽  
Author(s):  
Jia Yu ◽  
Chen Lai ◽  
Hoon Shim ◽  
Chengsong Xie ◽  
Lixin Sun ◽  
...  
Keyword(s):  
Motor Neurons ◽  
Genetic Ablation ◽  
Aged Mice ◽  
Spinal Motor Neurons ◽  
Spinal Motor

scholarly journals Relationship Between Excitability of Spinal Motor Neurons in Remote Muscles and Voluntary Movements

10.5772/67697 ◽  
2017 ◽  
Author(s):  
Naoki Kado ◽  
Yuki Takahashi ◽  
Satoshi Fujiwara ◽  
Masanori Ito
Keyword(s):  
Motor Neurons ◽  
Voluntary Movements ◽  
Spinal Motor Neurons ◽  
Spinal Motor

Hyperproliferation of synapses on spinal motor neurons of Duchenne muscular dystrophy and myotonic dystrophy patients

2003 ◽  
Vol 106 (6) ◽  
pp. 557-560 ◽  
Author(s):  
Masahiro Nagao ◽  
Shuichi Kato ◽  
Hideaki Hayashi ◽  
Hidemi Misawa
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Myotonic Dystrophy ◽  
Motor Neurons ◽  
Spinal Motor Neurons ◽  
Spinal Motor

scholarly journals MFN2 Deficiency Impairs Mitochondrial Transport and Downregulates Motor Protein Expression in Human Spinal Motor Neurons

2021 ◽  
Vol 14 ◽  
Author(s):  
Yongchao Mou ◽  
Joshua Dein ◽  
Zhenyu Chen ◽  
Mrunali Jagdale ◽  
Xue-Jun Li
Keyword(s):  
Protein Expression ◽  
Motor Neurons ◽  
Axonal Degeneration ◽  
Axonal Neuropathy ◽  
Motor Protein ◽  
Mitochondrial Morphology ◽  
Mitochondrial Transport ◽  
Spinal Motor Neurons ◽  
Spinal Motor ◽  
Hesc Lines

Charcot-Marie-Tooth (CMT) disease is one of the most common genetically inherited neurological disorders and CMT type 2A (CMT 2A) is caused by dominant mutations in the mitofusin-2 (MFN2) gene. MFN2 is located in the outer mitochondrial membrane and is a mediator of mitochondrial fusion, with an essential role in maintaining normal neuronal functions. Although loss of MFN2 induces axonal neuropathy, the detailed mechanism by which MFN2 deficiency results in axonal degeneration of human spinal motor neurons remains largely unknown. In this study, we generated MFN2-knockdown human embryonic stem cell (hESC) lines using lentivirus expressing MFN2 short hairpin RNA (shRNA). Using these hESC lines, we found that MFN2 loss did not affect spinal motor neuron differentiation from hESCs but resulted in mitochondrial fragmentation and dysfunction as determined by live-cell imaging. Notably, MFN2-knockodwn spinal motor neurons exhibited CMT2A disease-related phenotypes, including extensive perikaryal inclusions of phosphorylated neurofilament heavy chain (pNfH), frequent axonal swellings, and increased pNfH levels in long-term cultures. Importantly, MFN2 deficit impaired anterograde and retrograde mitochondrial transport within axons, and reduced the mRNA and protein levels of kinesin and dynein, indicating the interfered motor protein expression induced by MFN2 deficiency. Our results reveal that MFN2 knockdown induced axonal degeneration of spinal motor neurons and defects in mitochondrial morphology and function. The impaired mitochondrial transport in MFN2-knockdown spinal motor neurons is mediated, at least partially, by the altered motor proteins, providing potential therapeutic targets for rescuing axonal degeneration of spinal motor neurons in CMT2A disease.

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