A cellular model for Friedreich Ataxia reveals small-molecule glutathione peroxidase mimetics as novel treatment strategy.

@article{Jauslin2002ACM,
  title={A cellular model for Friedreich Ataxia reveals small-molecule glutathione peroxidase mimetics as novel treatment strategy.},
  author={Matthias L. Jauslin and Thomas Wirth and Thomas Meier and Fabrice Schoumacher},
  journal={Human molecular genetics},
  year={2002},
  volume={11 24},
  pages={3055-63}
}
Friedreich Ataxia (FRDA), the most prevalent of the inherited ataxias, is a multi-systemic disease with loss of sensory neurons and life-threatening hypertrophic cardiomyopathy as its most severe manifestations. Reduced levels of the mitochondrial protein frataxin lead to cell-damaging oxidative stress and consequently FRDA is considered as a model for more common neurodegenerative disorders in which reactive radicals and oxidative stress are involved. We have developed a cellular assay system… CONTINUE READING
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