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

被引：40

作者：

Angebault C. ^{[1
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Gueguen N. ^{[1
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Desquiret-Dumas V. ^{[1
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,4
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Chevrollier A. ^{[1
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,3
,4
]}

Guillet V. ^{[3
]}

Verny C. ^{[1
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,3
,5
]}

Cassereau J. ^{[1
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Ferre M. ^{[1
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Milea D. ^{[1
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]}

Amati-Bonneau P. ^{[1
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,4
]}

Bonneau D. ^{[1
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,4
]}

Procaccio V. ^{[1
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Reynier P. ^{[1
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Loiseau D. ^{[1
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,4
]}

机构：

[1] INSERM U771

[2] CNRS 6214

[3] LUNAM Université

[4] Département de Biochimie et Génétique, Centre Hospitalier Universitaire

[5] Département de Neurologie, Centre Hospitalier Universitaire

[6] Département d'Ophtalmologie, Centre Hospitalier Universitaire

[7] Département de Biochimie et Génétique, CHU d'Angers

来源：

BMC Research Notes | / 4卷 / 1期

关键词：

Rotenone; Mitochondrial Respiratory Chain; Idebenone; Respiratory Chain Activity; Polarographic Measurement;

D O I：

10.1186/1756-0500-4-557

中图分类号：

学科分类号：

摘要：

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.

引用

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