| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 10795932 | Biochimica et Biophysica Acta (BBA) - Bioenergetics | 2012 | 7 Pages |
Abstract
⺠The mitochondrial effects of idebenone in living cells are poorly characterized. ⺠Idebenone can be toxic or beneficial depending on its oxidation-reduction state. ⺠Idebenone is toxic through opening of the permeability transition pore. ⺠Idebenol stimulates respiration and ATP synthesis in cells lacking complex I. ⺠Short-chain quinones can be developed for the treatment of complex I defects.
Keywords
MELASMitochondrial encephalomyopathy with lactic acidosis and stroke-like episodesMOPSTMRMOCRNQO1LHONΔΨmFCCPPTPDTTDMEM4-morpholinepropanesulfonic acidDulbecco's modified Eagle's mediumNAD(P)H:quinone oxidoreductase 1ROSPermeability transition poreelectron transferPermeability transitionIdebenonedithiothreitolATP synthesisCyclosporincalcium retention capacitytetramethylrhodamine methyl esterMitochondriaOxygen consumption rateLeber's hereditary optic neuropathyCRCcarbonylcyanide-p-trifluoromethoxyphenyl hydrazoneReactive oxygen species
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Authors
Valentina Giorgio, Valeria Petronilli, Anna Ghelli, Valerio Carelli, Michela Rugolo, Giorgio Lenaz, Paolo Bernardi,