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- W2038404823 abstract "Idebenone [2,3-dimethoxy-5-methyl-6(10-hydroxydecyl)-1,4-benzoquinone] is a synthetic short-chain analogue of coenzyme Q10 (CoQ10). A variety of quinones (including idebenone) have also been shown to affect the mitochondrial permeability transition pore (PTP), a high-conductance inner membrane channel modulated by the proton electrochemical gradient and by many signaling molecules. The PTP links oxidative stress to cell death, and may be involved in the pathogenesis of Leber's hereditary optic neuropathy (LHON) and possibly to other conditions with complex I deficiency. Given these complex effects of idebenone, we have investigated its effects on bioenergetics and PTP modulation in intact cells. Our results indicate that: (i) idebenone promotes CsA-sensitive opening of the PTP and subsequent loss of pyridine nucleotides; (ii) dithiothreitol prevents PTP opening and its detrimental consequences; (iii) idebenol does not cause PTP opening, and stimulates electron transfer at complex III of the respiratory chain; and (iv) idebenol-stimulated respiration is coupled to ATP synthesis both in rotenone-treated normal cells and in RJ206 cells (harboring the 3460/ND1 LHON mutation) and XTC.UC1 thyroid oncocytoma cells (bearing a disruptive frameshift mutation in the MT-ND1 gene, which impairs complex I assembly). Thus, under proper experimental conditions idebenol, can be a useful tool to bypass complex I defiencies." @default.
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- W2038404823 date "2011-02-01" @default.
- W2038404823 modified "2023-09-26" @default.
- W2038404823 title "The Effects of Idebenone on Mitochondrial and Cellular Bioenergetics" @default.
- W2038404823 doi "https://doi.org/10.1016/j.bpj.2010.12.450" @default.
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