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Open Access Research article

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

Claire Angebault123, Naïg Gueguen1234, Valérie Desquiret-Dumas1234, Arnaud Chevrollier1234, Virginie Guillet3, Christophe Verny1235, Julien Cassereau1235, Marc Ferre1234, Dan Milea1236, Patrizia Amati-Bonneau1234, Dominique Bonneau1234, Vincent Procaccio1234, Pascal Reynier12347* and Dominique Loiseau1234

Author Affiliations

1 INSERM U771, Angers 49000, France

2 CNRS 6214, Angers 49000, France

3 LUNAM Université, Angers 49000, France

4 Département de Biochimie et Génétique, Centre Hospitalier Universitaire, Angers 49000, France

5 Département de Neurologie, Centre Hospitalier Universitaire, Angers 49000, France

6 Département d'Ophtalmologie, Centre Hospitalier Universitaire, Angers 49000, France

7 Département de Biochimie et Génétique, CHU d'Angers, 49933 Angers, France

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BMC Research Notes 2011, 4:557  doi:10.1186/1756-0500-4-557

Published: 22 December 2011

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.