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Lookup NU author(s): Dr Patrick Yu Wai Man,
Dr Florence Burte
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© 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.
Author(s): Yu-Wai-Man P, Soiferman D, Moore DG, Burte F, Saada A
Publication type: Article
Publication status: Published
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.
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