The idebenone metabolite QS10 restores electron transfer in complex I and coenzyme Q defects

Valentina Giorgio, Marco Schiavone, Chiara Galber, Marco Carini, Tatiana Da Ros, Valeria Petronilli, Francesco Argenton, Valerio Carelli, Manuel J. Acosta Lopez, Leonardo Salviati, Maurizio Prato, Paolo Bernardi, Biochimica et Biophysica Acta (BBA) - Bioenergetics, Available online 22 April 2018, ISSN 0005-2728, doi:10.1016/j.bbabio.2018.04.006.

Here we have studied the effects of quinones generated during in vivo metabolism of idebenone with specific emphasis on 6-(9-carboxynonyl)-2,3-dimethoxy-5-methyl-1,4-benzoquinone (QS10). QS10 partially restored respiration in cells deficient of complex I or of CoQ without inducing the mitochondrial permeability transition, a detrimental effect of idebenone that may offset its potential benefits.