Evaluating the therapeutic potential of idebenone and related quinone analogues in Leber hereditary optic neuropathy.
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.
['Yu-Wai-Man|Patrick|P|', 'Soiferman|Devorah|D|', 'Moore|David G|DG|', 'Burté|Florence|F|', 'Saada|Ann|A|']
D000255:Adenosine Triphosphate D000293:Adolescent D000975:Antioxidants D002478:Cells, Cultured D004734:Energy Metabolism D005347:Fibroblasts D006801:Humans D008297:Male D008875:Middle Aged D029242:Optic Atrophy, Hereditary, Leber D011809:Quinones D017382:Reactive Oxygen Species D014451:Ubiquinone D055815:Young Adult
Idebenone Quinone Leber hereditary optic neuropathy Coenzyme Q(10) Mitochondrial disease Optic atrophy