Open Access Repository

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

Downloads

Downloads per month over past year

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 ORCID: 0000-0002-5123-5999, Trounce, IA and Pebay, A 2017 , 'Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy' , Aging, vol. 9, no. 4 , pp. 1341-1350 , doi: 10.18632/aging.101231.

[img]
Preview
PDF
124228 Journal ...pdf | Download (1MB)

| Preview
[img]
Preview
PDF
124228 - Mitoch...pdf | Download (2MB)

| Preview

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.

Item Type: Article
Authors/Creators:Wong, RCB and Lim, SY and Hung, SSC and Jackson, S and Khan, S and Van Bergen, NJ and De Smit, E and Liang, HH and Kearns, LS and Clarke, L and Mackey, DA and Hewitt, AW and Trounce, IA and Pebay, A
Keywords: Leber’s hereditary optic neuropathy, cybrid, disease model, induced pluripotent stem cells, retinal ganglion cells
Journal or Publication Title: Aging
Publisher: Impact Journals LLC
ISSN: 1945-4589
DOI / ID Number: 10.18632/aging.101231
Copyright Information:

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

Related URLs:
Item Statistics: View statistics for this item

Actions (login required)

Item Control Page Item Control Page
TOP