The AMPA receptor antagonist perampanel robustly rescues amyotrophic lateral sclerosis (ALS) pathology in sporadic ALS model mice

@inproceedings{Akamatsu2016TheAR,
  title={The AMPA receptor antagonist perampanel robustly rescues amyotrophic lateral sclerosis (ALS) pathology in sporadic ALS model mice},
  author={Megumi Akamatsu and Takenari Yamashita and Naoki Hirose and Sayaka Teramoto and Shin Kwak},
  booktitle={Scientific reports},
  year={2016}
}
Both TDP-43 pathology and failure of RNA editing of AMPA receptor subunit GluA2, are etiology-linked molecular abnormalities that concomitantly occur in the motor neurons of the majority of patients with amyotrophic lateral sclerosis (ALS). AR2 mice, in which an RNA editing enzyme adenosine deaminase acting on RNA 2 (ADAR2) is conditionally knocked out in the motor neurons, exhibit a progressive ALS phenotype with TDP-43 pathology in the motor neurons through a Ca(2+)-permeable AMPA receptor… CONTINUE READING

Citations

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ALSUntangled 48: Perampanel (Fycompa).

  • Rick Bedlack
  • Medicine
  • Amyotrophic lateral sclerosis & frontotemporal degeneration
  • 2019
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Calpain-Dependent Degradation of Nucleoporins Contributes to Motor Neuron Death in a Mouse Model of Chronic Excitotoxicity.

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ADAR2 mislocalization and widespread RNA editing aberrations in C9orf72-mediated ALS/FTD

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