scholarly journals Changes in the Expression of FUS/TLS in Spinal Cords of SOD1 G93A Transgenic Mice and Correlation with Motor-Neuron Degeneration

2016 ◽  
Vol 12 (10) ◽  
pp. 1181-1190 ◽  
Author(s):  
Jiao Li ◽  
Yi Lu ◽  
Huiting Liang ◽  
Chunyan Tang ◽  
Lei Zhu ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Motor Neuron ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
G93a Transgenic Mice

Topiramate protects against motor neuron degeneration in organotypic spinal cord cultures but not in G93A SOD1 transgenic mice

2003 ◽  
Vol 338 (2) ◽  
pp. 107-110 ◽  
Author(s):  
Nicholas J. Maragakis ◽  
Mandy Jackson ◽  
Raquelli Ganel ◽  
Jeffrey D. Rothstein
Keyword(s):  
Spinal Cord ◽  
Transgenic Mice ◽  
Motor Neuron ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
G93a Sod1

scholarly journals S4-04-02: Overexpression of Wild-type TDP-43 Leads to Motor Neuron Degeneration and Spastic Quadriplegia in Germline Transgenic Mice

2009 ◽  
Vol 5 (4S_Part_5) ◽  
pp. e4-e4 ◽  
Author(s):  
Samir Kumar-Singh ◽  
Hans Wils ◽  
Gernot Kleinberger ◽  
Jonathan Janssens ◽  
Ivy Cuijt ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Motor Neuron ◽  
Wild Type ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Spastic Quadriplegia

scholarly journals Androgen Receptor YAC Transgenic Mice Recapitulate SBMA Motor Neuronopathy and Implicate VEGF164 in the Motor Neuron Degeneration

Neuron ◽  
2004 ◽  
Vol 41 (5) ◽  
pp. 687-699 ◽  
Author(s):  
Bryce L Sopher ◽  
Patrick S Thomas ◽  
Michelle A LaFevre-Bernt ◽  
Ida E Holm ◽  
Scott A Wilke ◽  
...  
Keyword(s):  
Androgen Receptor ◽  
Transgenic Mice ◽  
Motor Neuron ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration

scholarly journals Overexpression of ferroptosis defense enzyme Gpx4 retards motor neuron disease of SOD1G93A mice

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Liuji Chen ◽  
Ren Na ◽  
Kirsten Danae McLane ◽  
Cody Sylvester Thompson ◽  
Ju Gao ◽  
...  
Keyword(s):  
Lipid Peroxidation ◽  
Transgenic Mice ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Neurons ◽  
Disease Onset ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Sporadic Als

AbstractDegeneration and death of motor neurons in Amyotrophic Lateral Sclerosis (ALS) are associated with increased lipid peroxidation. Lipid peroxidation is the driver of ferroptosis, an iron-dependent oxidative mode of cell death. However, the importance of ferroptosis in motor neuron degeneration of ALS remains unclear. Glutathione peroxidase 4 (Gpx4) is a key enzyme in suppressing ferroptosis by reducing phospholipid hydroperoxides in membranes. To assess the effect of increased protection against ferroptosis on motor neuron disease, we generated SOD1G93AGPX4 double transgenic mice by cross-breeding GPX4 transgenic mice with SOD1G93A mice, a widely used ALS mouse model. Compared with control SOD1G93A mice, both male and female SOD1G93AGPX4 mice had extended lifespans. SOD1G93AGPX4 mice also showed delayed disease onset and increased motor function, which were correlated with ameliorated spinal motor neuron degeneration and reduced lipid peroxidation. Moreover, cell toxicity induced by SOD1G93A was ameliorated by Gpx4 overexpression and by chemical inhibitors of ferroptosis in vitro. We further found that the anti-ferroptosis defense system in spinal cord tissues of symptomatic SOD1G93A mice and sporadic ALS patients might be compromised due to deficiency of Gpx4. Thus, our results suggest that ferroptosis plays a key role in motor neuron degeneration of ALS.

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