Elianne P Bulthuis, Claudia Einer, Felix Distelmaier, Laszlo Groh, Sjenet E van Emst-de Vries, Els van de Westerlo, Melissa van de Wal, Jori Wagenaars, Richard J Rodenburg, Jan A M Smeitink, Niels P Riksen, Peter H G M Willems, Merel J W Adjobo-Hermans, Hans Zischka, Werner J H Koopman
Attachment of cargo molecules to lipophilic triphenylphosphonium (TPP+ ) cations is a widely applied strategy for mitochondrial targeting. We previously demonstrated that the vitamin E-derived antioxidant Trolox increases the levels of active mitochondrial complex I (CI), the first complex of the electron transport chain (ETC), in primary human skin fibroblasts (PHSFs) of Leigh Syndrome (LS) patients with isolated CI deficiency. Primed by this finding, we here studied the cellular effects of mitochondria-targeted Trolox (MitoE10), mitochondria-targeted ubiquinone (MitoQ10) and their mitochondria-targeting moiety decylTPP (C10 -TPP+ )...
August 1, 2022: Free Radical Biology & Medicine