MAPT-A152T mutation drives neuronal hyperactivity through Fyn-NMDAR signaling in human iPSC-Derived neurons: Insights into Alzheimer's pathogenesis

Introduction: Tau protein plays a pivotal role in the pathogenesis of Alzheimer's disease (AD) and in regulating neuronal excitability. Among tau-coding microtubule associated protein tau (MAPT) gene mutations, the A152T mutation is reported to increase the risk of AD and neuronal excitability...

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Main Authors: Maika Itsuno, Hirokazu Tanabe, Etsuko Sano, Takashi Sasaki, Chisato Oyama, Hiroko Bannai, Koichi Saito, Kazuhiko Nakata, Setsu Endoh-Yamagami, Hideyuki Okano, Sumihiro Maeda
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Language:English
Published: Elsevier 2025-03-01
Series:Regenerative Therapy
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Online Access:http://www.sciencedirect.com/science/article/pii/S2352320424002281
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author Maika Itsuno
Hirokazu Tanabe
Etsuko Sano
Takashi Sasaki
Chisato Oyama
Hiroko Bannai
Koichi Saito
Kazuhiko Nakata
Setsu Endoh-Yamagami
Hideyuki Okano
Sumihiro Maeda
author_facet Maika Itsuno
Hirokazu Tanabe
Etsuko Sano
Takashi Sasaki
Chisato Oyama
Hiroko Bannai
Koichi Saito
Kazuhiko Nakata
Setsu Endoh-Yamagami
Hideyuki Okano
Sumihiro Maeda
author_sort Maika Itsuno
collection DOAJ
description Introduction: Tau protein plays a pivotal role in the pathogenesis of Alzheimer's disease (AD) and in regulating neuronal excitability. Among tau-coding microtubule associated protein tau (MAPT) gene mutations, the A152T mutation is reported to increase the risk of AD and neuronal excitability in mouse models. Methods: To investigate the effects of MAPT gene expression and its mutations on neuronal activity in human neurons, we employed genome editing technology to introduce the A152T or P301S mutations into induced pluripotent stem cells (iPSCs). We then differentiated them into excitatory and inhibitory neurons. As a control, iPSCs in which the MAPT gene was replaced with a fluorescent protein were also created. Results: In excitatory neuronal cultures, the A152T mutation was found to enhance spontaneous neuronal activity and the association of tau and Fyn. However, in inhibitory neuron-enriched cultures, the A152T mutation did not affect neuronal activity. Inhibition of NMDA receptors (NMDAR) and the reduction of tau protein levels decreased neuronal excitability in both A152T/A152T and healthy control (WT/WT) excitatory neurons. In addition, the A152T mutation increased the interaction between tau and Fyn. These findings suggest that the tau-Fyn interaction plays a critical role in regulating neuronal activity under physiological conditions, while the A152T mutation enhances neuronal activity by strengthening this endogenous interaction between tau and Fyn. In addition, transcriptomic analysis revealed structural changes specific to excitatory neurons with the A152T mutation. Common changes observed in both A152T and P301S lines recapitulated a dedifferentiation phenotype, consistent with previous reports. Conclusions: These data demonstrate that the A152T mutation in the MAPT gene increases neuronal excitability through the tau-Fyn-NMDAR pathway in excitatory neurons, shedding light on its role in AD pathogenesis.
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spelling doaj-art-57b7b8a562ec41e3a49f8118c86de3d92025-08-20T02:58:29ZengElsevierRegenerative Therapy2352-32042025-03-012820121310.1016/j.reth.2024.12.009MAPT-A152T mutation drives neuronal hyperactivity through Fyn-NMDAR signaling in human iPSC-Derived neurons: Insights into Alzheimer's pathogenesisMaika Itsuno0Hirokazu Tanabe1Etsuko Sano2Takashi Sasaki3Chisato Oyama4Hiroko Bannai5Koichi Saito6Kazuhiko Nakata7Setsu Endoh-Yamagami8Hideyuki Okano9Sumihiro Maeda10Department of Physiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, JapanFUJIFILM Corporation, Bio Science & Engineering Laboratories, 577 Ushijima, Kaisei-cho, Ashigarakami-gun, Kanagawa 258-8577, JapanDepartment of Physiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan; Keio University Regenerative Medicine Research Center (KRM), 3-25-10 Tonomachi, Kawasaki-ku, Kawasaki, Kanagawa 210-0821, JapanCenter for Supercentenarian Medical Research, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, JapanDepartment of Electrical Engineering and Biosciences, School of Advanced Science and Engineering, Waseda University, 2-2 Wakamatsu-cho, Shinjuku, Tokyo 162-0056, JapanDepartment of Electrical Engineering and Biosciences, School of Advanced Science and Engineering, Waseda University, 2-2 Wakamatsu-cho, Shinjuku, Tokyo 162-0056, JapanFUJIFILM Corporation, Bio Science & Engineering Laboratories, 577 Ushijima, Kaisei-cho, Ashigarakami-gun, Kanagawa 258-8577, JapanFUJIFILM Corporation, Bio Science & Engineering Laboratories, 577 Ushijima, Kaisei-cho, Ashigarakami-gun, Kanagawa 258-8577, JapanFUJIFILM Corporation, Bio Science & Engineering Laboratories, 577 Ushijima, Kaisei-cho, Ashigarakami-gun, Kanagawa 258-8577, Japan; Corresponding author.Department of Physiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan; Keio University Regenerative Medicine Research Center (KRM), 3-25-10 Tonomachi, Kawasaki-ku, Kawasaki, Kanagawa 210-0821, Japan; Corresponding author. Department of Physiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan.Department of Physiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan; Corresponding author.Introduction: Tau protein plays a pivotal role in the pathogenesis of Alzheimer's disease (AD) and in regulating neuronal excitability. Among tau-coding microtubule associated protein tau (MAPT) gene mutations, the A152T mutation is reported to increase the risk of AD and neuronal excitability in mouse models. Methods: To investigate the effects of MAPT gene expression and its mutations on neuronal activity in human neurons, we employed genome editing technology to introduce the A152T or P301S mutations into induced pluripotent stem cells (iPSCs). We then differentiated them into excitatory and inhibitory neurons. As a control, iPSCs in which the MAPT gene was replaced with a fluorescent protein were also created. Results: In excitatory neuronal cultures, the A152T mutation was found to enhance spontaneous neuronal activity and the association of tau and Fyn. However, in inhibitory neuron-enriched cultures, the A152T mutation did not affect neuronal activity. Inhibition of NMDA receptors (NMDAR) and the reduction of tau protein levels decreased neuronal excitability in both A152T/A152T and healthy control (WT/WT) excitatory neurons. In addition, the A152T mutation increased the interaction between tau and Fyn. These findings suggest that the tau-Fyn interaction plays a critical role in regulating neuronal activity under physiological conditions, while the A152T mutation enhances neuronal activity by strengthening this endogenous interaction between tau and Fyn. In addition, transcriptomic analysis revealed structural changes specific to excitatory neurons with the A152T mutation. Common changes observed in both A152T and P301S lines recapitulated a dedifferentiation phenotype, consistent with previous reports. Conclusions: These data demonstrate that the A152T mutation in the MAPT gene increases neuronal excitability through the tau-Fyn-NMDAR pathway in excitatory neurons, shedding light on its role in AD pathogenesis.http://www.sciencedirect.com/science/article/pii/S2352320424002281Alzheimer's disease (AD)Induced pluripotent stem cells (iPSCs)hTau-A152T mutationTau-Fyn-NMDAR pathway
spellingShingle Maika Itsuno
Hirokazu Tanabe
Etsuko Sano
Takashi Sasaki
Chisato Oyama
Hiroko Bannai
Koichi Saito
Kazuhiko Nakata
Setsu Endoh-Yamagami
Hideyuki Okano
Sumihiro Maeda
MAPT-A152T mutation drives neuronal hyperactivity through Fyn-NMDAR signaling in human iPSC-Derived neurons: Insights into Alzheimer's pathogenesis
Regenerative Therapy
Alzheimer's disease (AD)
Induced pluripotent stem cells (iPSCs)
hTau-A152T mutation
Tau-Fyn-NMDAR pathway
title MAPT-A152T mutation drives neuronal hyperactivity through Fyn-NMDAR signaling in human iPSC-Derived neurons: Insights into Alzheimer's pathogenesis
title_full MAPT-A152T mutation drives neuronal hyperactivity through Fyn-NMDAR signaling in human iPSC-Derived neurons: Insights into Alzheimer's pathogenesis
title_fullStr MAPT-A152T mutation drives neuronal hyperactivity through Fyn-NMDAR signaling in human iPSC-Derived neurons: Insights into Alzheimer's pathogenesis
title_full_unstemmed MAPT-A152T mutation drives neuronal hyperactivity through Fyn-NMDAR signaling in human iPSC-Derived neurons: Insights into Alzheimer's pathogenesis
title_short MAPT-A152T mutation drives neuronal hyperactivity through Fyn-NMDAR signaling in human iPSC-Derived neurons: Insights into Alzheimer's pathogenesis
title_sort mapt a152t mutation drives neuronal hyperactivity through fyn nmdar signaling in human ipsc derived neurons insights into alzheimer s pathogenesis
topic Alzheimer's disease (AD)
Induced pluripotent stem cells (iPSCs)
hTau-A152T mutation
Tau-Fyn-NMDAR pathway
url http://www.sciencedirect.com/science/article/pii/S2352320424002281
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