Poster Open Access

Characterization of the activity-dependent development of IPSC-derived neurons from Fragile X patients

Maussion, Gilles; Rocha, Cecilia; Soubannier, Vincent

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  "publisher": "Zenodo", 
  "DOI": "10.5281/zenodo.3515460", 
  "language": "eng", 
  "title": "Characterization of the activity-dependent development of IPSC-derived neurons from Fragile X patients", 
  "issued": {
    "date-parts": [
  "abstract": "<p>Fragile X syndrome is a form of syndromic autism whose genetic causes have been relatively well uncovered. It is<br>\nactually mainly caused by a CGG triplet expansion in the 5&rsquo; UTR sequence of FMR1 gene, affecting mostly men. FMR1<br>\nencodes a mRNA binding protein which is involved in the regulation of local translation at the synaptic level. The<br>\nmechanisms leading from such gene mutations to a neurodevelopmental disorder still need to be investigated. While<br>\nseveral studies have shown that the neuronal development is driven by cellular activity and connectivity, we aim to<br>\nfurther investigate the effect of FMR1 repression on the neuronal activity taking advantage of IPSC-derived neurons<br>\nfrom patient&rsquo;s cells. IPSC-derived neurons will be investigated through calcium imaging to characterized their pattern<br>\nof spontaneous activities, as well as their capability to respond to neurotransmitter through extra-synaptic receptors.<br>\nA multielectrode array approach is going to be used to analyse the overall network activities. Those studies should<br>\nprovide further information on the impairment of activity-dependent neuronal development in Fragile X syndrome.</p>", 
  "author": [
      "family": "Maussion, Gilles"
      "family": "Rocha, Cecilia"
      "family": "Soubannier, Vincent"
  "version": "V1.0", 
  "type": "graphic", 
  "id": "3515460"
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