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 - 10.1186/1756-0500-4-557
DO - 10.1186/1756-0500-4-557
M3 - Journal article
C2 - 22192149
SN - 1756-0500
VL - 4
SP - 557
JO - B M C Research Notes
JF - B M C Research Notes
IS - 1
ER -