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.

journal_name

Aging (Albany NY)

journal_title

Aging

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,Pébay A

doi

10.18632/aging.101231

subject

Has Abstract

pub_date

2017-04-01 00:00:00

pages

1341-1350

issue

4

issn

1945-4589

pii

101231

journal_volume

9

pub_type

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