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

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posted on 2023-05-19, 15:54 authored by Wong, RCB, Lim, SY, Hung, SSC, Jackson, S, Khan, S, Van Bergen, NJ, De Smit, E, Liang, HH, Kearns, LS, Clarke, L, David MackeyDavid Mackey, Alexander HewittAlexander Hewitt, Trounce, IA, Pebay, A
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.

History

Publication title

Aging

Volume

9

Issue

4

Pagination

1341-1350

ISSN

1945-4589

Department/School

Tasmanian School of Medicine

Publisher

Impact Journals LLC

Place of publication

United States

Rights statement

Copyright 2017 the authors. Licensed under a Creative Commons Attribution 3.0 License (CC BY 3.0). https://creativecommons.org/licenses/by/3.0/

Repository Status

  • Open

Socio-economic Objectives

Clinical health not elsewhere classified

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    University Of Tasmania

    Categories

    Keywords

    Leber’s hereditary optic neuropathycybriddisease modelinduced pluripotent stem cellsretinal ganglion cells

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    In Copyright

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