Mitochondrial impairment and synaptic dysfunction are associated with neurological defects in iPSCs-derived cortical neurons of MERRF patients

Mitochondrial impairment and synaptic dysfunction are associated with neurological defects in iPSCs-derived cortical neurons of MERRF patients

Abstract Background Myoclonic epilepsy with ragged-red fibers (MERRF) syndrome is a rare inherited mitochondrial disease mainly caused by the m.8344A > G mutation in mitochondrial tRNALys gene, and usually manifested as complex neurological disorders and muscle weakness. Currently, the pathogenic...

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Bibliographic Details
Main Authors: Yu-Ting Wu, Hui-Yi Tay, Jung-Tse Yang, Hsiao-Hui Liao, Yi-Shing Ma, Yau-Huei Wei
Format: Article
Language:English
Published: BMC 2023-08-01
Series:Journal of Biomedical Science
Subjects:
AMPARs
Disease modeling
Electrophysiological activity
Excitatory neurons
iPSCs
MERRF syndrome
Online Access:https://doi.org/10.1186/s12929-023-00966-8

Internet

https://doi.org/10.1186/s12929-023-00966-8

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