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

Claire Angebault; Naig Gueguen; Valerie Desquiret-Dumas; Arnaud Chevrollier; Virgine Guillet; Christophe Verny; Julien Cassereau; Marc Ferre; Dan Milea; Patrizia Amati-Bonneau; Dominique Bonneau; Vincent Procaccio; Pascal Reynier; Dominique Loiseau

doi:http://dx.doi.org/10.1186/1756-0500-4-557

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

Claire Angebault, Naig Gueguen, Valerie Desquiret-Dumas, Arnaud Chevrollier, Virgine Guillet, Christophe Verny, Julien Cassereau, Marc Ferre, Dan Milea, Patrizia Amati-Bonneau, Dominique Bonneau, Vincent Procaccio, Pascal Reynier, Dominique Loiseau

40 Citations (Scopus)

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.

Original language	English
Journal	BMC Research Notes
Volume	4
Issue number	1
Pages (from-to)	557
ISSN	1756-0500
DOIs	https://doi.org/10.1186/1756-0500-4-557
Publication status	Published - 2011

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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(1), 557. https://doi.org/10.1186/1756-0500-4-557

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, vol. 4, no. 1, pp. 557. https://doi.org/10.1186/1756-0500-4-557

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title = "Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration",

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.",

author = "Claire Angebault and Naig Gueguen and Valerie Desquiret-Dumas and Arnaud Chevrollier and Virgine Guillet and Christophe Verny and Julien Cassereau and Marc Ferre and Dan Milea and Patrizia Amati-Bonneau and Dominique Bonneau and Vincent Procaccio and Pascal Reynier and Dominique Loiseau",

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doi = "http://dx.doi.org/10.1186/1756-0500-4-557",

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TY - JOUR

T1 - Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration

AU - Angebault, Claire

AU - Gueguen, Naig

AU - Desquiret-Dumas, Valerie

AU - Chevrollier, Arnaud

AU - Guillet, Virgine

AU - Verny, Christophe

AU - Cassereau, Julien

AU - Ferre, Marc

AU - Milea, Dan

AU - Amati-Bonneau, Patrizia

AU - Bonneau, Dominique

AU - Procaccio, Vincent

AU - Reynier, Pascal

AU - Loiseau, Dominique

PY - 2011

Y1 - 2011

N2 - 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.

AB - 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.

U2 - http://dx.doi.org/10.1186/1756-0500-4-557

DO - http://dx.doi.org/10.1186/1756-0500-4-557

M3 - Journal article

SN - 1756-0500

VL - 4

SP - 557

JO - BMC Research Notes

JF - BMC Research Notes

IS - 1

ER -

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

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