Effects of tocotrienol supplementation in Friedreich’s ataxia: A model of oxidative stress pathology

Alessandra Bolotta, Antonella Pini, Provvidenza M Abruzzo, Alessandro Ghezzo, Alessandra Modesti, Tania Gamberi, Carla Ferreri, Francesca Bugamelli, Filippo Fortuna, Silvia Vertuani, Stefano Manfredini, Cinzia Zucchini, Marina Marini; Experimental Biology and Medicine. Dec. 2019, doi:10.1177/1535370219890873.

Oxidative stress is involved in the pathogenesis of Friedreich's ataxia (FRDA), a genetic disorder causing neurodegeneration due to the dramatic reduction in the expression of frataxin. To date, no cure is available for FRDA patients. In some countries, FRDA patients assume idebenone in order to counteract the effects of frataxin deficiency. We demonstrate that idebenone treatment alone is not able to abrogate oxidative stress in FRDA patients, whereas the combined treatment with tocotrienols might be more efficient and perhaps produce clinical improvement. In fact, a decrease in oxidative stress and inflammation markers can be seen after two months and is more pronounced after one year of treatment. This is, in our opinion, valuable information for clinicians, since idebenone is the treatment of choice for FRDA patients in some countries.

Effects of tocotrienol supplementation in Friedreich’s ataxia: A model of oxidative stress pathology