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

Intisar, Aseer; Kang, Hyun Gyu; Kim, Woon‐Hae; Kim, Min Young; Kim, Yu Seon; Mo, Yun Jeoung; Lee, Yun‐Il; Kim, Minseok S.

doi:10.1016/j.bios.2022.115055

Cited by 2 publications

(1 citation statement)

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“…Alternatively, AWG may stimulate other physiological gap junction channels composed of different connexin isoforms to rectify the abnormal channel properties caused by gene mutation [13]. Interestingly, electrical stimulation (ES) at 20 Hz for 1 h has been shown to ameliorate intracellular PMP22 (peripheral myelin protein 22) aggregation and return the PMP22 distribution to healthy conditions in PMP22overexpressing Schwannoma cells in an in vitro model of CMT1A [14]. The authors suggested that enhanced gene Mathews Journal of Case Reports https://doi.org/10.30654/MJCR.10118 expression of myelin basic protein (MBP), myelin-associated glycoprotein (MAG), myelination-regulating transcription factors Krox-20, Oct-6, c-Jun, and Sox10 provided mechanistic insight into the efficacy of ES [14].…”

Section: Discussionmentioning

confidence: 99%

Cytokine-Induced Neurogenesis in Charcot-Marie-Tooth neuropathy with Connexin 32 Gene Mutation

Shirasawa

2023

MJCR

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Charcot-Marie-Tooth (CMT) neuropathy is one of the most common neuromuscular disorders. Despite the identification of more than 100 causative genes, there are few therapeutic options. We present the case of a 66-year-old man carrying a CMT mutation in the connexin 32 gene with the APOE genotype ε4/ε3 that had exhibited symptoms of slowly progressive muscle weakness and atrophy in the hands and feet and recently developed memory impairment. The muscle weakness, motor dysfunction, and memory impairment symptoms were significantly improved by cytokine-induced neurogenesis combined with physical wave therapy using an arbitrary waveform generator (AWG). Here, we discuss the possible mechanistic basis of how this new treatment ameliorates the symptoms of CMT and propose a novel clinical treatment strategy for CMT.

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Section: Discussionmentioning

confidence: 99%

Cytokine-Induced Neurogenesis in Charcot-Marie-Tooth neuropathy with Connexin 32 Gene Mutation

Shirasawa

2023

MJCR

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The prospect of electroceutical intervention and its implementation toward intractable neuromuscular diseases

Intisar,

Kim

2024

Current Opinion in Biomedical Engineering

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

Cited by 2 publications

References 81 publications

Cytokine-Induced Neurogenesis in Charcot-Marie-Tooth neuropathy with Connexin 32 Gene Mutation

Cytokine-Induced Neurogenesis in Charcot-Marie-Tooth neuropathy with Connexin 32 Gene Mutation

The prospect of electroceutical intervention and its implementation toward intractable neuromuscular diseases

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