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

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 variab

SUBMITTER: Angebault C 

PROVIDER: S-EPMC3285568 | biostudies-literature | 2011 Dec

REPOSITORIES: biostudies-literature