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Open AccessReview

Progress of Induced Pluripotent Stem Cell Technologies to Understand Genetic Epilepsy

1
Department of Experimental Medicine, University of Genova, Viale Benedetto XV, 3, 16132 Genoa, Italy
2
Center for Synaptic Neuroscience and Technology, Istituto Italiano di Tecnologia, Largo Rosanna Benzi 10, 16132 Genoa, Italy
3
Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Largo P. Daneo 3, 16132 Genoa, Italy
4
Unità Operativa Complessa Genetica Medica, Istituto di Ricovero e Cura a Carattere Scientifico Giannina Gaslini, Genova Italy, Via G. Gaslini 5, 16147 Genoa, Italy
5
Istituto di Ricovero e Cura a Carattere Scientifico, Ospedale Policlinico San Martino, Largo Rosanna Benzi 10, 16132 Genoa, Italy
*
Authors to whom correspondence should be addressed.
These authors contributed equally to this work.
Int. J. Mol. Sci. 2020, 21(2), 482; https://doi.org/10.3390/ijms21020482
Received: 10 December 2019 / Revised: 8 January 2020 / Accepted: 10 January 2020 / Published: 12 January 2020
(This article belongs to the Special Issue From hIPSCs to Adult Cells in a Dish: Promises and Pitfalls)
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. View Full-Text
Keywords: epilepsy; induced pluripotent stem cells; disease modeling; cerebral organoid; neuronal excitability; transplantation epilepsy; induced pluripotent stem cells; disease modeling; cerebral organoid; neuronal excitability; transplantation
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MDPI and ACS Style

Sterlini, B.; Fruscione, F.; Baldassari, S.; Benfenati, F.; Zara, F.; Corradi, A. Progress of Induced Pluripotent Stem Cell Technologies to Understand Genetic Epilepsy. Int. J. Mol. Sci. 2020, 21, 482.

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