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Electroceutical approach ameliorates intracellular PMP22 aggregation and promotes pro-myelinating pathways in a CMT1A in vitro model

Title
Electroceutical approach ameliorates intracellular PMP22 aggregation and promotes pro-myelinating pathways in a CMT1A in vitro model
Author(s)
Intisar, AseerWoo, HanwoongKang, Hyun GyuKim, Woon-HaeShin, Hyun YoungKim, Min YoungKim, Yu SeonMo, Yun JeoungLee, Yun-IlKim, Minseok S.
Issued Date
2023-03
Citation
Biosensors and Bioelectronics, v.224
Type
Article
Author Keywords
ElectroceuticalsBioelectronic deviceHigh-throughput screeningPeripheral myelin protein 22Schwannoma cellCMT1A
Keywords
MARIE-TOOTH DISEASEELECTRIC-FIELD STIMULATIONSCHWANN-CELLSREGENERATIONFREQUENCYACCUMULATIONACTIVATIONOUTGROWTHCALNEXINALTERS
ISSN
0956-5663
Abstract
Charcot-Marie-Tooth disease subtype 1A (CMT1A) is one of the most prevalent demyelinating peripheral neuropathies worldwide, caused by duplication of the peripheral myelin protein 22 (PMP22) gene, which is expressed primarily in Schwann cells (SCs). PMP22 overexpression in SCs leads to intracellular aggregation of the protein, which eventually results in demyelination. Unfortunately, previous biochemical approaches have not resulted in an approved treatment for CMT1A disease, compelling the pursuit for a biophysical approach such as electrical stimulation (ES). However, the effects of ES on CMT1A SCs have remained unexplored. In this study, we established PMP22-overexpressed Schwannoma cells as a CMT1A in vitro model, and investigated the biomolecular changes upon applying ES via a custom-made high-throughput ES platform, screening for the condition that delivers optimal therapeutic effects. While PMP22-overexpressed Schwannoma exhibited intracellular PMP22 aggregation, ES at 20 Hz for 1 h improved this phenomenon, bringing PMP22 distribution closer to healthy condition. ES at this condition also enhanced the expression of the genes encoding myelin basic protein (MBP) and myelin-associated glycoprotein (MAG), which are essential for assembling myelin sheath. Furthermore, ES altered the gene expression for myelination-regulating transcription factors Krox-20, Oct-6, c-Jun and Sox10, inducing pro-myelinating effects in PMP22-overexpressed Schwannoma. While electroceuticals has previously been applied in the peripheral nervous system towards acquired peripheral neuropathies such as pain and nerve injury, this study demonstrates its effectiveness towards ameliorating biomolecular abnormalities in an in vitro model of CMT1A, an inherited peripheral neuropathy. These findings will facilitate the clinical translation of an electroceutical treatment for CMT1A. © 2023 Elsevier B.V.
URI
http://hdl.handle.net/20.500.11750/46226
DOI
10.1016/j.bios.2022.115055
Publisher
Pergamon Press Ltd.
Related Researcher
  • 이윤일 Lee, Yun-Il 바이오융합연구부
  • Research Interests
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Appears in Collections:
Division of Biotechnology 1. Journal Articles
Department of New Biology BioDr. Lab - Nanobiomedicine 1. Journal Articles

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