艾地苯醌可提高Leber遗传性视神经病变（LHON）患者成纤维细胞中的线粒体复合物I活性，同时对呼吸作用产生相反的影响。
Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration.
作者信息
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
机构信息
INSERM U771, Angers 49000, France.
出版信息
BMC Res Notes. 2011 Dec 22;4:557. doi: 10.1186/1756-0500-4-557.
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.
背景
Leber遗传性视神经病变（LHON）由呼吸链复合体I亚基的突变引起。自1992年以来，患者一直使用艾地苯醌进行治疗，但其疗效仍存在争议。
方法
我们使用酶促和极谱测量法评估了艾地苯醌对LHON患者成纤维细胞的作用。
结果
与对照组相比，经治疗的成纤维细胞中复合体I活性高42%（p = 0.002）。尽管复合体I活性有所改善，但对线粒体呼吸的影响却相互矛盾，在某些情况下导致呼吸受损，而在另一些情况下则起到刺激作用。
结论
这些结果表明，艾地苯醌能够弥补LHON患者细胞中的复合体I缺陷，但对呼吸的影响各不相同，这表明患者从该治疗中获益的可能性可能并不相同。
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