Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question.

@article{Armstrong2010DoesOS,
  title={Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question.},
  author={Jeffrey Armstrong and Omar M. Khdour and Sidney M Hecht},
  journal={FASEB journal : official publication of the Federation of American Societies for Experimental Biology},
  year={2010},
  volume={24 7},
  pages={2152-63}
}
Friedreich's ataxia (FRDA) is a hereditary neurodegenerative disease that frequently culminates in cardiac failure at an early age. FRDA is believed to arise from reduced synthesis of the mitochondrial iron chaperone frataxin due to impaired gene transcription, which leads to mitochondrial iron accumulation, dysfunction of mitochondrial Fe-S containing enzymes, and increased Fenton-mediated free radical production. Recent reports have challenged this generally accepted hypothesis, by suggesting… CONTINUE READING