Friedreich Ataxia: current state-of-the-art, and future prospects for mitochondrial-focused therapies

Federico V. Pallardó, Giovanni Pagano, Laura R. Rodríguez, Pilar Gonzalez-Cabo, Alex Lyakhovich, Marco Trifuoggi; Translational Research,
2020, doi:10.1016/j.trsl.2020.08.009.

The present mini review is aimed at evaluating the basic and clinical reports on the roles and the use of a set of iron chelators, antioxidants and some cofactors involved in the key mitochondrial functions. Extensive literature has focused on the protective roles of iron chelators, coenzyme Q10 and analogs, and vitamin E, altogether with varying outcomes in clinical studies. Other studies have suggested mitoprotective roles for other mitochondrial cofactors, involved in Krebs cycle, such as alpha-lipoic acid and carnitine, involved in acyl transport across the mitochondrial membrane.


Friedreich Ataxia: current state-of-the-art, and future prospects for mitochondrial-focused therapies