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

Raymond C B Wong, Shiang Y Lim, Sandy S C Hung, Stacey Jackson, Shahnaz Khan, Nicole J Van Bergen, Elisabeth De Smit, Helena Liang, Lisa S Kearns, Linda Clarke, David A Mackey, Alex W Hewitt, Ian A. Trounce, Alice Pébay

Research output: Contribution to journalArticle

24 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1341-1350
Number of pages10
JournalAging
Volume9
Issue number4
DOIs
Publication statusPublished - Apr 2017

Fingerprint Dive into the research topics of 'Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy'. Together they form a unique fingerprint.

Cite this