INaP selective inhibition reverts precocious inter- and motorneurons hyperexcitability in the Sod1-G93R zebrafish ALS model

@inproceedings{Benedetti2016INaPSI,
  title={INaP selective inhibition reverts precocious inter- and motorneurons hyperexcitability in the Sod1-G93R zebrafish ALS model},
  author={Lorena Gabriela Benedetti and Anna Ghilardi and Elsa Rottoli and Marcella De Maglie and Laura Prosperi and Carla Perego and Mirko Baruscotti and Annalisa Bucchi and Luca Del Giacco and Maura Francolini},
  booktitle={Scientific reports},
  year={2016}
}
The pathogenic role of SOD1 mutations in amyotrophic lateral sclerosis (ALS) was investigated using a zebrafish disease model stably expressing the ALS-linked G93R mutation. In addition to the main pathological features of ALS shown by adult fish, we found remarkably precocious alterations in the development of motor nerve circuitry and embryo behavior, and suggest that these alterations are prompted by interneuron and motor neuron hyperexcitability triggered by anomalies in the persistent… CONTINUE READING
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