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Evaluating the therapeutic potential of idebenone and related quinone analogues in Leber hereditary optic neuropathy

Lookup NU author(s): Dr Patrick Yu Wai Man, David Moore, Dr Florence Burte

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This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


Abstract

© 2017 The Authors. Leber hereditary optic neuropathy (LHON) is an important cause of mitochondrial blindness among young adults. In this study, we investigated the potential of four quinone analogues (CoQ1, CoQ10, decylubiquinone and idebenone) in compensating for the deleterious effect of the m.11778G>A mitochondrial DNA mutation. The LHON fibroblast cell lines tested exhibited reduced cell growth, impaired mitochondrial bioenergetics and elevated levels of reactive oxygen species (ROS). Idebenone increased ATP production and reduced ROS levels, but the effect was partial and cell-specific. The remaining quinone analogues had variable effects and a negative impact on certain mitochondrial parameters was observed in some cell lines.


Publication metadata

Author(s): Yu-Wai-Man P, Soiferman D, Moore DG, Burte F, Saada A

Publication type: Article

Publication status: Published

Journal: Mitochondrion

Year: 2017

Volume: 36

Pages: 36-42

Print publication date: 01/09/2017

Online publication date: 16/01/2017

Acceptance date: 12/01/2017

Date deposited: 12/05/2017

ISSN (print): 1567-7249

ISSN (electronic): 1872-8278

Publisher: Elsevier B.V.

URL: http://doi.org/10.1016/j.mito.2017.01.004

DOI: 10.1016/j.mito.2017.01.004


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