eCite Digital Repository
Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy
Citation
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]
 | PDF 3Mb |
Copyright Statement
Copyright 2017 the authors. Licensed under a Creative Commons Attribution 3.0 License (CC BY 3.0). https://creativecommons.org/licenses/by/3.0/
DOI: doi: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 Details
| Item Type: | Refereed Article |
|---|---|
| Keywords: | Leber’s hereditary optic neuropathy, cybrid, disease model, induced pluripotent stem cells, retinal ganglion cells |
| Research Division: | Medical and Health Sciences |
| Research Group: | Ophthalmology and Optometry |
| Research Field: | Ophthalmology |
| Objective Division: | Health |
| Objective Group: | Clinical Health (Organs, Diseases and Abnormal Conditions) |
| Objective Field: | Hearing, Vision, Speech and Their Disorders |
| 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: | 10 |
| Deposited By: | Menzies Institute for Medical Research |
| Deposited On: | 2018-02-14 |
| Last Modified: | 2018-06-06 |
| Downloads: | 41 View Download Statistics |
Repository Staff Only: item control page