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Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy


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, Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy, Aging, 9, (4) pp. 1341-1350. ISSN 1945-4589 (2017) [Refereed Article]


Copyright Statement

Copyright 2017 the authors. Licensed under a Creative Commons Attribution 3.0 License (CC BY 3.0).

DOI: doi:10.18632/aging.101231


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 Details

Item Type:Refereed Article
Keywords:Leberís hereditary optic neuropathy, cybrid, disease model, induced pluripotent stem cells, retinal ganglion cells
Research Division:Biomedical and Clinical Sciences
Research Group:Ophthalmology and optometry
Research Field:Ophthalmology
Objective Division:Health
Objective Group:Clinical health
Objective Field:Clinical health not elsewhere classified
UTAS Author:Mackey, DA (Professor David Mackey)
UTAS Author:Hewitt, AW (Professor Alex Hewitt)
ID Code:124228
Year Published:2017
Web of Science® Times Cited:32
Deposited By:Menzies Institute for Medical Research
Deposited On:2018-02-14
Last Modified:2022-08-26
Downloads:98 View Download Statistics

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