Elena Gammella, Stefania Recalcati, and Gaetano Cairo. Oxidative Medicine and Cellular Longevity, Volume 2016 (2016), Article ID 8629024, 9 pages, doi:10.1155/2016/8629024
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Abundant evidence shows that a number of neurodegenerative disorders are characterized by regional iron accumulation in particular areas of the central and/or peripheral nervous systems.
This is often caused by cellular iron redistribution and may result in iron-catalyzed Fenton chemistry.Friedrich's ataxia (FRDA) is a paradigmatic example because the disruption of iron homeostasis in this disease has been well defined. The disease results from loss of function mutations (most often triplet expansion) in the FXN gene that lead to decreased expression of frataxin, a mitochondrial iron-binding protein that interacts with proteins involved in the mitochondrial Fe-S cluster biogenesis. In patients, frataxin deficiency results in disruption of Fe-S cluster biosynthesis, severe mitochondrial iron overload, a hallmark of Fe-S defects, and increased sensitivity to oxidative stress.
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