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.
Abbreviations
- Cybrid
- cyb
- induced pluripotent stem cells
- iPSCs
- Leber hereditary optic neuropathy
- LHON
- Mitochondrial Encephalomyopathy Lactic Acidosis and Stroke-like episodes
- MELAS
- mitochondrial
- mt
- retinal ganglion cells
- RGCs
Copyright
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