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

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Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration. / Angebault, Claire; Gueguen, Naig; Desquiret-Dumas, Valerie; Chevrollier, Arnaud; Guillet, Virgine; Verny, Christophe; Cassereau, Julien; Ferre, Marc; Milea, Dan; Amati-Bonneau, Patrizia; Bonneau, Dominique; Procaccio, Vincent; Reynier, Pascal; Loiseau, Dominique.

In: BMC Research Notes, Vol. 4, No. 1, 2011, p. 557.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

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

APA

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

Vancouver

Angebault C, Gueguen N, Desquiret-Dumas V, Chevrollier A, Guillet V, Verny C et al. Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration. BMC Research Notes. 2011;4(1):557. https://doi.org/10.1186/1756-0500-4-557

Author

Angebault, Claire ; Gueguen, Naig ; Desquiret-Dumas, Valerie ; Chevrollier, Arnaud ; Guillet, Virgine ; Verny, Christophe ; Cassereau, Julien ; Ferre, Marc ; Milea, Dan ; Amati-Bonneau, Patrizia ; Bonneau, Dominique ; Procaccio, Vincent ; Reynier, Pascal ; Loiseau, Dominique. / Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration. In: BMC Research Notes. 2011 ; Vol. 4, No. 1. pp. 557.

Bibtex

@article{7e2e67679bc2465489a39f397e3f47de,
title = "Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration",
abstract = "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",
year = "2011",
doi = "http://dx.doi.org/10.1186/1756-0500-4-557",
language = "English",
volume = "4",
pages = "557",
journal = "BMC Research Notes",
issn = "1756-0500",
publisher = "BioMed Central Ltd.",
number = "1",

}

RIS

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 - 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.

AB - 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.

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

VL - 4

SP - 557

JO - BMC Research Notes

JF - BMC Research Notes

SN - 1756-0500

IS - 1

ER -

ID: 40179698