Article
INaP selective inhibition reverts precocious inter- and motorneurons hyperexcitability in the Sod1-G93R zebrafish ALS model.
Scientific reports - 15 Apr 2016
Benedetti Lorena, Ghilardi Anna, Rottoli Elsa, De Maglie Marcella, Prosperi Laura, Perego Carla, Baruscotti Mirko, Bucchi Annalisa, Del Giacco Luca, Francolini Maura
Abstract excerpt
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...
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