Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy.

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.

SUBMITTER: Wong RCB 

PROVIDER: S-EPMC5425131 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature