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
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