scholarly journals In vivo optical imaging of motor neuron autophagy in a mouse model of amyotrophic lateral sclerosis

Autophagy ◽  
2011 ◽  
Vol 7 (9) ◽  
pp. 985-992 ◽  
Author(s):  
FengFeng Tian ◽  
Nobutoshi Morimoto ◽  
WenTao Liu ◽  
Yasuyuki Ohta ◽  
Kentaro Deguchi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Optical Imaging ◽  
In Vivo Optical Imaging ◽  
Lateral Sclerosis

scholarly journals LanCL1 promotes motor neuron survival and extends the lifespan of amyotrophic lateral sclerosis mice

2019 ◽  
Vol 27 (4) ◽  
pp. 1369-1382 ◽  
Author(s):  
Honglin Tan ◽  
Mina Chen ◽  
Dejiang Pang ◽  
Xiaoqiang Xia ◽  
Chongyangzi Du ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Neuronal Survival ◽  
Disease Onset ◽  
Alternative Mechanism ◽  
Specific Expression ◽  
Motor Neuron Survival ◽  
Lateral Sclerosis

Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive loss of motor neurons. Improving neuronal survival in ALS remains a significant challenge. Previously, we identified Lanthionine synthetase C-like protein 1 (LanCL1) as a neuronal antioxidant defense gene, the genetic deletion of which causes apoptotic neurodegeneration in the brain. Here, we report in vivo data using the transgenic SOD1G93A mouse model of ALS indicating that CNS-specific expression of LanCL1 transgene extends lifespan, delays disease onset, decelerates symptomatic progression, and improves motor performance of SOD1G93A mice. Conversely, CNS-specific deletion of LanCL1 leads to neurodegenerative phenotypes, including motor neuron loss, neuroinflammation, and oxidative damage. Analysis reveals that LanCL1 is a positive regulator of AKT activity, and LanCL1 overexpression restores the impaired AKT activity in ALS model mice. These findings indicate that LanCL1 regulates neuronal survival through an alternative mechanism, and suggest a new therapeutic target in ALS.

Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model

2009 ◽  
Vol 34 (3) ◽  
pp. 511-517 ◽  
Author(s):  
Bradley J. Turner ◽  
Nicholas J. Parkinson ◽  
Kay E. Davies ◽  
Kevin Talbot
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Survival Motor Neuron ◽  
Lateral Sclerosis

scholarly journals Serial in vivo imaging of transplanted allogeneic neural stem cell survival in a mouse model of amyotrophic lateral sclerosis

2017 ◽  
Vol 289 ◽  
pp. 96-102 ◽  
Author(s):  
Amit K. Srivastava ◽  
Sarah K. Gross ◽  
Akshata A. Almad ◽  
Camille A. Bulte ◽  
Nicholas J. Maragakis ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Stem Cell ◽  
Mouse Model ◽  
Cell Survival ◽  
Neural Stem Cell ◽  
In Vivo Imaging ◽  
Stem Cell Survival ◽  
Lateral Sclerosis
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