Glutamate receptors: RNA editing and death of motor neurons

@article{Kawahara2004GlutamateRR,
  title={Glutamate receptors: RNA editing and death of motor neurons},
  author={Yukio Kawahara and Kyoko Ito and Hui Sun and Hitoshi Aizawa and Ichiro Kanazawa and Shin Kwak},
  journal={Nature},
  year={2004},
  volume={427},
  pages={801-801}
}
The aetiology of sporadic amyotrophic lateral sclerosis (ALS), a fatal paralytic disease, is largely unknown. Here we show that there is a defect in the editing of the messenger RNA encoding the GluR2 subunit of glutamate AMPA receptors in the spinal motor neurons of individuals affected by ALS. This failure to swap an arginine for a glutamine residue at a crucial site in the subunit, which occurs normally in the affected brain areas of patients with other neurodegenerative diseases, will… 
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TLDR
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TLDR
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TLDR
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TLDR
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The AMPA receptor antagonist perampanel robustly rescues amyotrophic lateral sclerosis (ALS) pathology in sporadic ALS model mice
TLDR
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Exploration of the Pathogenesis of Amyotrophic Lateral Sclerosis from the Perspective of Motor Neuron TDP-43 Protein Expression and ADAR2 Activity
TLDR
Upregulating ADAR2 in mouse motor neurons using an adeno-associated virus serotype 9 vector that enables gene delivery to a wide array of central neurons after peripheral administration effectively prevented progressive motor dysfunction and rescued the motor neurons from death by normalizing TDP-43 expression.
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