Introduction to Special Issue on Mitochondrial Redox Signaling in Health and Disease

Juan P. Bolaños, Enrique Cadenas, Michael R. Duchen, Mark B. Hampton, Giovanni E. Mann, Michael P. Murphy, Free Radical Biology and Medicine, Available online 5 August 2016, ISSN 0891-5849, doi:10.1016/j.freeradbiomed.2016.08.004.

The genetic deficiency of the mitochondrial protein frataxin is the cause of Friedreich ataxia. Frataxin deficiency is not associated with cognitive impairment, but with increased oxidative stress with alterations in lipid metabolism, which are discussed as potential therapeutic approaches in Friedreich ataxia. The mitochondrial Lon protease is involved in several neurological disorders, such as hereditary Parkinson’s disease, Friedreich ataxia, familial amyotrophic lateral sclerosis, brain ischemia and stroke. In these disorders, the physiological functions of the Lon protease are altered: as a protease, as a chaperone,and as a mtDNA binding protein.