Disabled early recruitment of antioxidant defenses in Friedreich's ataxia.

@article{ChantrelGroussard2001DisabledER,
  title={Disabled early recruitment of antioxidant defenses in Friedreich's ataxia.},
  author={Karine Chantrel-Groussard and Vanna G{\'e}romel and H{\'e}l{\`e}ne M Puccio and Michel Koenig and Arnold Munnich and Agn{\'e}s R{\"o}tig and Pierre Rustin},
  journal={Human molecular genetics},
  year={2001},
  volume={10 19},
  pages={
          2061-7
        }
}
Friedreich's ataxia (FRDA) results from a generalized deficiency of mitochondrial iron-sulfur protein activity ascribed to mitochondrial iron overload. However, iron overload appears to be a late event in the disease. Here we show that neither superoxide dismutases nor the import iron machinery was induced by an endogenous oxidative stress in FRDA patients' fibroblasts in contrast to control cells. Superoxide dismutase activity was not induced in the heart of conditional frataxin-KO mice either… CONTINUE READING

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