Review Article Neurodegeneration in Friedreich’s Ataxia: From Defective Frataxin to Oxidative Stress

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Friedreich’s ataxia is the most common inherited autosomal recessive ataxia and is characterized by progressive degeneration of the peripheral and central nervous systems and cardiomyopathy. This disease is caused by the silencing of the FXN gene and reduced levels of the encoded protein, frataxin. Frataxin is a mitochondrial protein that functions primarily in iron-sulfur cluster synthesis. This small protein with a