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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, Shahnaz, 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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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
Authors/Creators:Wong, RCB and Lim, SY and Hung, SSC and Jackson, S and Khan, Shahnaz 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).

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