Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy
Raymond C B Wong1,2, Shiang Y Lim3, Sandy S C Hung1,2
1Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, East Melbourne, Australia.
Aging
|April 30, 2017
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.
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