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Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy
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Wong, RCB, Lim, SY, Hung, SSC, Jackson, S, Khan, S, Van Bergen, NJ, De Smit, E, Liang, HH, Kearns, LS, Clarke, L, Mackey, DA, Hewitt, AW
ORCID: 0000-0002-5123-5999, Trounce, IA and Pebay, A 2017
, 'Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy'
, Aging, vol. 9, no. 4
, pp. 1341-1350
, doi: 10.18632/aging.101231.

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Official URL: http://dx.doi.org/10.18632/aging.101231
Abstract
Cybrid technology was used to replace Leber hereditary optic neuropathy (LHON) causing mitochondrial DNA (mtDNA) mutations from patient-specific fibroblasts with wildtype mtDNA, and mutation-free induced pluripotent stem cells (iPSCs) were generated subsequently. Retinal ganglion cell (RGC) differentiation demonstrates increased cell death in LHON-RGCs and can be rescued in cybrid corrected RGCs.
Item Type: | Article |
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Authors/Creators: | Wong, RCB and Lim, SY and Hung, SSC and Jackson, S and Khan, S and Van Bergen, NJ and De Smit, E and Liang, HH and Kearns, LS and Clarke, L and Mackey, DA and Hewitt, AW and Trounce, IA and Pebay, A |
Keywords: | Leber’s hereditary optic neuropathy, cybrid, disease model, induced pluripotent stem cells, retinal ganglion cells |
Journal or Publication Title: | Aging |
Publisher: | Impact Journals LLC |
ISSN: | 1945-4589 |
DOI / ID Number: | 10.18632/aging.101231 |
Copyright Information: | Copyright 2017 the authors. Licensed under a Creative Commons Attribution 3.0 License (CC BY 3.0). https://creativecommons.org/licenses/by/3.0/ |
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