Please use this identifier to cite or link to this item: https://doi.org/10.1186/1756-0500-4-557
Title: Idebenone increases mitochondrial complex i activity in fibroblasts from LHON patients while producing contradictory effects on respiration
Authors: Angebault, C
Gueguen, N
Desquiret-Dumas, V
Chevrollier, A
Guillet, V
Verny, C
Cassereau, J
Ferre, M
Milea, D 
Amati-Bonneau, P
Bonneau, D
Procaccio, V
Reynier, P
Loiseau, D
Issue Date: 2011
Publisher: BMC
Citation: Angebault, C, Gueguen, N, Desquiret-Dumas, V, Chevrollier, A, Guillet, V, Verny, C, Cassereau, J, Ferre, M, Milea, D, Amati-Bonneau, P, Bonneau, D, Procaccio, V, Reynier, P, Loiseau, D (2011). Idebenone increases mitochondrial complex i activity in fibroblasts from LHON patients while producing contradictory effects on respiration. BMC Research Notes 4 : 557. ScholarBank@NUS Repository. https://doi.org/10.1186/1756-0500-4-557
Rights: Attribution 4.0 International
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. © 2011 Angebault et al; licensee BioMed Central Ltd.
Source Title: BMC Research Notes
URI: https://scholarbank.nus.edu.sg/handle/10635/178157
ISSN: 1756-0500
DOI: 10.1186/1756-0500-4-557
Rights: Attribution 4.0 International
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