Cell mitochondria use frataxin to make energy. Cells without enough frataxin can not produce energy efficiently. Toxins may build up and damage cells in a process known as oxidative stress. This effect is most pronounced in the cells of the peripheral nerves, spinal cord, brain, and heart. An additional theory states that a lack of frataxin causes increased iron in mitochondria. The excess mineral reacts with oxygen to form free radicals, which can destroy cells.

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