scholarly journals In Vivo NMDA Receptor Activation Accelerates Motor Unit Maturation, Protects Spinal Motor Neurons, and Enhances SMN2 Gene Expression in Severe Spinal Muscular Atrophy Mice

2010 ◽  
Vol 30 (34) ◽  
pp. 11288-11299 ◽  
Author(s):  
O. Biondi ◽  
J. Branchu ◽  
G. Sanchez ◽  
C. Lancelin ◽  
S. Deforges ◽  
...  
2002 ◽  
Vol 103 (5) ◽  
pp. 428-436 ◽  
Author(s):  
Róbert Adalbert ◽  
József Engelhardt ◽  
László Siklós

2011 ◽  
Vol 31 (9) ◽  
pp. 3328-3335 ◽  
Author(s):  
L.-q. Gong ◽  
L.-j. He ◽  
Z.-y. Dong ◽  
X.-h. Lu ◽  
M.-m. Poo ◽  
...  

Author(s):  
Kazuhide Asakawa ◽  
Hiroshi Handa ◽  
Koichi Kawakami

Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by progressive degeneration of motor neurons in the brain and spinal cord. Spinal motor neurons align along the spinal cord length within the vertebral column, and extend long axons to connect with skeletal muscles covering the body surface. Due to this anatomy, spinal motor neurons are among the most difficult cells to observe in vivo. Larval zebrafish have transparent bodies that allow non-invasive visualization of whole cells of single spinal motor neurons, from somas to the neuromuscular synapses. This unique feature, combined with its amenability to genome editing, pharmacology, and optogenetics, enables functional analyses of ALS-associated proteins in the spinal motor neurons in vivo with subcellular resolution. Here, we review the zebrafish skeletal neuromuscular system and the optical methods used to study it. We then introduce a recently developed optogenetic zebrafish ALS model that uses light illumination to control oligomerization, phase transition and aggregation of the ALS-associated DNA/RNA-binding protein called TDP-43. Finally, we will discuss how this disease-in-a-fish ALS model can help solve key questions about ALS pathogenesis and lead to new ALS therapeutics.


2021 ◽  
Author(s):  
Feng Wang ◽  
Xinya Fu ◽  
Meiemei Li ◽  
Xingran Wang ◽  
Jile Xie ◽  
...  

The loss of motor function in patients with spinal cord injury (SCI) is primarily due to the severing of the corticospinal tract (CST). Spinal motor neurons are located in the anterior horn of the spinal cord, and as the lower neurons of the CST, they control voluntary movement. Furthermore, its intrinsic axonal growth ability is significantly stronger than that of cerebral cortex pyramid neurons, which are the upper CST neurons. Therefore, we established an axonal regeneration model of spinal motor neurons to investigate the feasibility of repairing SCI by promoting axonal regeneration of spinal motor neurons. We demonstrated that conditionally knocking out pten in mature spinal motor neurons drastically enhanced axonal regeneration in vivo, and the regenerating axons of the spinal motor neurons re-established synapses with other cells in the damaged spinal cord. Thus, this strategy may serve as a novel and effective treatment method for SCI.


1997 ◽  
Vol 18 (2) ◽  
pp. 149-158 ◽  
Author(s):  
J. OWENS ◽  
D. J. WYPER ◽  
J. PATTERSON ◽  
D. R.P. BROWN ◽  
A. T. ELLIOTT ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document