| Autor: |
Angebault Claire, Gueguen Naïg, Desquiret-Dumas Valérie, Chevrollier Arnaud, Guillet Virginie, Verny Christophe, Cassereau Julien, Ferre Marc, Milea Dan, Amati-Bonneau Patrizia, Bonneau Dominique, Procaccio Vincent, Reynier Pascal, Loiseau Dominique |
| Jazyk: |
angličtina |
| Rok vydání: |
2011 |
| Předmět: |
|
| Zdroj: |
BMC Research Notes, Vol 4, Iss 1, p 557 (2011) |
| Druh dokumentu: |
article |
| ISSN: |
1756-0500 |
| DOI: |
10.1186/1756-0500-4-557 |
| Popis: |
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. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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