Specific deletion of NaV1.1 sodium channels in inhibitory interneurons causes seizures and premature death in a mouse model of Dravet syndrome.

@article{Cheah2012SpecificDO,
  title={Specific deletion of NaV1.1 sodium channels in inhibitory interneurons causes seizures and premature death in a mouse model of Dravet syndrome.},
  author={Christine S. Cheah and Frank H. Yu and Ruth E. Westenbroek and Franck K Kalume and John C. Oakley and Gregory B Potter and John L. Rubenstein and William A. Catterall},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2012},
  volume={109 36},
  pages={14646-51}
}
Heterozygous loss-of-function mutations in the brain sodium channel Na(V)1.1 cause Dravet syndrome (DS), a pharmacoresistant infantile-onset epilepsy syndrome with comorbidities of cognitive impairment and premature death. Previous studies using a mouse model of DS revealed reduced sodium currents and impaired excitability in GABAergic interneurons in the hippocampus, leading to the hypothesis that impaired excitability of GABAergic inhibitory neurons is the cause of epilepsy and premature… CONTINUE READING
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Effect of deletion of Nav1.1 channels on sodium current in dissociated neocortical interneurons in a mouse model of severe myoclonic epilepsy in infancy

  • Y Abe, F Kalume, RE Westenbroek, T Scheuer, WA Catterall
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Reduced excitability of GABAergic interneurons in the reticular nucleus of the thalamus and sleep impairment in a mouse model of Severe Myoclonic Epilepsy of Infancy

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