Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells.

@article{Jiralerspong2001ManganeseSD,
  title={Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells.},
  author={Sao Jiralerspong and Bixia Ge and Thomas J. Hudson and Massimo Pandolfo},
  journal={FEBS letters},
  year={2001},
  volume={509 1},
  pages={101-5}
}
Iron-mediated oxidative stress has been implicated in the pathology of the neurodegenerative disease Friedreich ataxia (FRDA). Here, we show that normal upregulation of the stress defense protein manganese superoxide dismutase (MnSOD) fails to occur in FRDA fibroblasts exposed to iron. This impaired induction was observed at iron levels in which increased activation of the redox-sensitive factor NF-kappaB was absent. Furthermore, MnSOD induction could only be partially suppressed by… CONTINUE READING

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