Progress of induced pluripotent stem cell technologies to understand genetic epilepsy

Bruno Sterlini, Floriana Fruscione, Simona Baldassari, Fabio Benfenati, Federico Zara, Anna Corradi

Research output: Contribution to journalReview articlepeer-review

Abstract

The study of the pathomechanisms by which gene mutations lead to neurological diseases has benefit from several cellular and animal models. Recently, induced Pluripotent Stem Cell (iPSC) technologies have made possible the access to human neurons to study nervous system disease-related mechanisms, and are at the forefront of the research into neurological diseases. In this review, we will focalize upon genetic epilepsy, and summarize the most recent studies in which iPSC-based technologies were used to gain insight on the molecular bases of epilepsies. Moreover, we discuss the latest advancements in epilepsy cell modeling. At the two dimensional (2D) level, single-cell models of iPSC-derived neurons lead to a mature neuronal phenotype, and now allow a reliable investigation of synaptic transmission and plasticity. In addition, functional characterization of cerebral organoids enlightens neuronal network dynamics in a three-dimensional (3D) structure. Finally, we discuss the use of iPSCs as the cutting-edge technology for cell therapy in epilepsy.

Original languageEnglish
Article number482
JournalInternational Journal of Molecular Sciences
Volume21
Issue number2
DOIs
Publication statusPublished - Jan 2 2020

Keywords

  • Cerebral organoid
  • Disease modeling
  • Epilepsy
  • Induced pluripotent stem cells
  • Neuronal excitability
  • Transplantation

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry

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