TY - JOUR
T1 - Progress of induced pluripotent stem cell technologies to understand genetic epilepsy
T2 - International Journal of Molecular Sciences
AU - Sterlini, B.
AU - Fruscione, F.
AU - Baldassari, S.
AU - Benfenati, F.
AU - Zara, F.
AU - Corradi, A.
N1 - Cited By :2
Export Date: 26 March 2021
Correspondence Address: Zara, F.; Department of Neurosciences, Largo P. Daneo 3, Italy; email: FedericoZara@gaslini.org
PY - 2020/1/2
Y1 - 2020/1/2
N2 - 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.
AB - 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.
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U2 - 10.3390/ijms21020482
DO - 10.3390/ijms21020482
M3 - Review article
C2 - 31940887
VL - 21
JO - Int. J. Mol. Sci.
JF - Int. J. Mol. Sci.
SN - 1661-6596
IS - 2
M1 - 482
ER -