Nrf2 activation in the treatment of neurodegenerative diseases: a focus on its role in mitochondrial bioenergetics and function

Noemí Esteras, Albena T. Dinkova-Kostova, Andrey Y. Abramov; Biological Chemistry. ISSN (Online) 1437-4315, ISSN (Print) 1431-6730, DOI: 10.1515/hsz-2015-0295, January 2016

Currently, REATA Pharmaceuticals has initiated a clinical trial with a novel Nrf2 activator, named RTA 408, for the treatment of Friedreich’s ataxia (ClinicalTrials.gov, NCT02255435). This neurodegenerative disease is caused by deficiency of the protein frataxin, which causes the disruption of iron-sulphur cluster biosynthesis, mitochondrial iron overload and an increased sensitivity to oxidative stress, mainly due to a decrease in the expression of Nrf2.