Across Dimensions: Developing 2D and 3D Human iPSC-Based Models of Fragile X Syndrome
Abstract
:1. Introduction
1.1. Fragile X Syndrome
1.2. Pluripotent Stem Cells (PSCs) and Translational Applications
2. Two-Dimensional (2D) Human iPSC-Based Cellular Models of FXS
2.1. Generation of Human iPSC-Derived Cellular Models of FXS
2.2. Gene Expression and Translation in the 2D hiPSC-Derived Cell Models of FXS
2.3. Studies of FMRP Function and Targets
Reference | Section(s) of Review | Model Type(s) | Summary of FXS-iPSC-Relevant Findings |
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Urbach et al. (2010) [46] | Generation of human iPSC-derived cellular models of FXS
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Sheridan et al. (2011) [47] | Generation of human iPSC-derived cellular models of FXS
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Doers et al. (2014) [48] | Generation of human iPSC-derived cellular models of FXS
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Esanov et al. (2016) [49] | Generation of human iPSC-derived cellular models of FXS
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De Esch et al. (2014) [50] | Generation of human iPSC-derived cellular models of FXS
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Brykczynska et al. (2016) [51] | Generation of human iPSC-derived cellular models of FXS
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Halevy et al. (2015) [52] | Gene expression and translation in the 2D hiPSC-derived cell models of FXS |
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Utami et al. (2020) [53] | Gene expression and translation in the 2D hiPSC-derived cell models of FXS Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS |
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Lu et al. (2016) [54] | Gene expression and translation in the 2D hiPSC-derived cell models of FXS |
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Boland et al. (2017) [55] | Gene expression and translation in the 2D hiPSC-derived cell models of FXS Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS |
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Sunamura et al. (2018) [56] | Gene expression and translation in the 2D hiPSC-derived cell models of FXS |
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Raj et al. (2021) [21] | Gene expression and translation in the 2D hiPSC-derived cell models of FXS Modeling FXS with human brain organoids |
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Kurosaki et al. (2021) [57] | Gene expression and translation in the 2D hiPSC-derived cell models of FXS |
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Li et al. (2020) [23] | Studies of FMRP function and targets |
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Goering et al. (2020) [58] | Studies of FMRP function and targets |
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Niedringhaus et al. (2015) [90] | Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS |
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Zhang et al. (2018) [60] | Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS |
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Das Sharma et al. (2020) [61] | Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS |
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Danesi et al. (2018) [63] | Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS |
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Achuta et al. (2017) [62] | Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS |
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Achuta et al. (2018) [59] | Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS
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Brighi et al. (2021) [24] | Characterization of neurodevelopmental and electrophysiological abnormalities of iPSC-derived cell models of FXS
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Bar-Nur et al. (2012) [64] | FMR1 reactivation—Pharmacological rescue |
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Kaufmann et al. (2015) [65] | FMR1 reactivation—Pharmacological rescue |
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Kumari et al. (2015) [67] | FMR1 reactivation—Pharmacological rescue |
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Li et al. (2017) [68] | FMR1 reactivation—Pharmacological rescue |
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Vershkov et al. (2019) [69] | FMR1 reactivation—Pharmacological rescue |
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Kumari et al. (2020) [66] | FMR1 reactivation—Pharmacological rescue |
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Park et al. (2015) [70] | FMR1 reactivation—Gene editing |
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Xie et al. (2016) [71] | FMR1 reactivation—Gene editing |
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Liu et al. (2018) [72] | FMR1 reactivation—Gene editing |
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Graef et al. (2020) [73] | FMR1 reactivation—Gene editing |
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Kang et al. (2021) [22] | Modeling FXS with human brain organoids |
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2.4. Characterization of Neurodevelopmental and Electrophysiological Abnormalities of iPSC-Derived Cell Models of FXS
2.5. Reactivation of FMR1 Gene in FXS
2.5.1. FMR1 Reactivation—Pharmacological Rescue
2.5.2. FMR1 Reactivation—Gene Editing
3. Three-Dimensional (3D) hiPSC-Derived Models of FXS
3.1. Brain Organoids—In Vitro System Recapitulating Human Neurodevelopment
3.2. Modeling FXS with Human Brain Organoids
4. Limitations and Future Directions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Lee, A.; Xu, J.; Wen, Z.; Jin, P. Across Dimensions: Developing 2D and 3D Human iPSC-Based Models of Fragile X Syndrome. Cells 2022, 11, 1725. https://0-doi-org.brum.beds.ac.uk/10.3390/cells11111725
Lee A, Xu J, Wen Z, Jin P. Across Dimensions: Developing 2D and 3D Human iPSC-Based Models of Fragile X Syndrome. Cells. 2022; 11(11):1725. https://0-doi-org.brum.beds.ac.uk/10.3390/cells11111725
Chicago/Turabian StyleLee, Azalea, Jie Xu, Zhexing Wen, and Peng Jin. 2022. "Across Dimensions: Developing 2D and 3D Human iPSC-Based Models of Fragile X Syndrome" Cells 11, no. 11: 1725. https://0-doi-org.brum.beds.ac.uk/10.3390/cells11111725