Transcriptional Dysregulation and Impaired Neuronal Activity in <i>FMR1</i> Knock-Out and Fragile X Patients’ iPSC-Derived Models

Transcriptional Dysregulation and Impaired Neuronal Activity in <i>FMR1</i> Knock-Out and Fragile X Patients’ iPSC-Derived Models

Fragile X syndrome (FXS) is caused by a repression of the <i>FMR1</i> gene that codes the Fragile X mental retardation protein (FMRP), an RNA binding protein involved in processes that are crucial for proper brain development. To better understand the consequences of the absence of FMRP,...

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Bibliographic Details
Main Authors: Gilles Maussion, Cecilia Rocha, Narges Abdian, Dimitri Yang, Julien Turk, Dulce Carrillo Valenzuela, Luisa Pimentel, Zhipeng You, Barbara Morquette, Michael Nicouleau, Eric Deneault, Samuel Higgins, Carol X.-Q. Chen, Wolfgang E. Reintsch, Stanley Ho, Vincent Soubannier, Sarah Lépine, Zora Modrusan, Jessica Lund, William Stephenson, Rajib Schubert, Thomas M. Durcan
Format: Article
Language:English
Published: MDPI AG 2023-10-01
Series:International Journal of Molecular Sciences
Subjects:
iPSC-derived neurons
RNA transcriptomics
multielectrode array
activity-dependent development
Fragile X syndrome
cortical neurons
Online Access:https://www.mdpi.com/1422-0067/24/19/14926

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https://www.mdpi.com/1422-0067/24/19/14926

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