TY - DATA T1 - Erratum: Functional Recovery of Human Cells Harbouring the Mitochondrial DNA Mutation MERRF A8344G via Peptide-Mediated Mitochondrial Delivery PY - 2017/07/25 AU - Chang J.-C. AU - Liu K.-H. AU - Li Y.-C. AU - Kou S.-J. AU - Wei Y.-H. AU - Chuang C.-S. AU - Hsieh M. AU - Liu C.-S. UR - https://karger.figshare.com/articles/dataset/Erratum_Functional_Recovery_of_Human_Cells_Harbouring_the_Mitochondrial_DNA_Mutation_MERRF_b_i_A8344G_i_b_via_Peptide-Mediated_Mitochondrial_Delivery/5241466 DO - 10.6084/m9.figshare.5241466.v1 L4 - https://ndownloader.figshare.com/files/8956102 L4 - https://ndownloader.figshare.com/files/8956105 KW - Cell-penetrating peptide KW - Mitochondrial delivery KW - Myoclonic epilepsy with ragged-red fibres syndrome KW - Mitochondrial functions KW - Mitochondrial biogenesis KW - Mitochondrial fusion/fission proteins N2 - We explored the feasibility of mitochondrial therapy using the cell-penetrating peptide Pep-1 to transfer mitochondrial DNA (mtDNA) between cells and rescue a cybrid cell model of the mitochondrial disease myoclonic epilepsy with ragged-red fibres (MERRF) syndrome. Pep-1-conjugated wild-type mitochondria isolated from parent cybrid cells incorporating a mitochondria-specific tag were used as donors for mitochondrial delivery into MERRF cybrid cells (MitoB2) and mtDNA-depleted Rho-zero cells (Mitoρ°). Forty-eight hours later, translocation of Pep-1-labelled mitochondria into the mitochondrial regions of MitoB2 and Mitoρ° host cells was observed (delivery efficiencies of 77.48 and 82.96%, respectively). These internalized mitochondria were maintained for at least 15 days in both cell types and were accompanied by mitochondrial function recovery and cell survival by preventing mitochondria-dependent cell death. Mitochondrial homeostasis analyses showed that peptide-mediated mitochondrial delivery (PMD) also increased mitochondrial biogenesis in both cell types, but through distinct regulatory pathways involving mitochondrial dynamics. Dramatic decreases in mitofusin-2 (MFN2) and dynamin-related protein 1/fission 1 were observed in MitoB2 cells, while Mitoρ° cells showed a significant increase in optic atrophy 1 and MFN2. These findings suggest that PMD can be used as a potential therapeutic intervention for mitochondrial disorders. ER -