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Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration.

Abstract : UNLABELLED: ABSTRACT: BACKGROUND: Leber's hereditary optic neuropathy (LHON) is caused by mutations in the complex I subunits of the respiratory chain. Although patients have been treated with idebenone since 1992, the efficacy of the drug is still a matter of debate. METHODS: We evaluated the effect of idebenone in fibroblasts from LHON patients using enzymatic and polarographic measurements. RESULTS: Complex I activity was 42% greater in treated fibroblasts compared to controls (p = 0.002). Despite this complex I activity improvement, the effects on mitochondrial respiration were contradictory, leading to impairment in some cases and stimulation in others. CONCLUSION: These results indicate that idebenone is able to compensate the complex I deficiency in LHON patient cells with variable effects on respiration, indicating that the patients might not be equally likely to benefit from the treatment.
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https://www.hal.inserm.fr/inserm-00673659
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Submitted on : Thursday, February 23, 2012 - 9:06:10 PM
Last modification on : Wednesday, November 13, 2019 - 9:30:03 AM
Long-term archiving on: : Friday, November 23, 2012 - 2:30:39 PM

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Claire Angebault, Naïg Gueguen, Valérie Desquiret-Dumas, Arnaud Chevrollier, Virginie Guillet, et al.. Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration.. BMC Research Notes, BioMed Central, 2011, 4 (1), pp.557. ⟨10.1186/1756-0500-4-557⟩. ⟨inserm-00673659⟩

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