| Title: | Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy |
| Authors: | 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 Trounce, IA Pebay, A |
| Issue Year: | 2017 |
| Publisher | IMPACT JOURNALS LLC |
| Series | Aging-US: |
| 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. |
| URI: | https://publications.svi.edu.au/publications/6251 |
| Other Identifiers | 10.18632/aging.101231 |
| Publication type | Article |