Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial iron.

@article{Richardson2001DevelopmentOP,
  title={Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial iron.},
  author={Des. R. Richardson and C Mouralian and Prem Ponka and Elisabeth Becker},
  journal={Biochimica et biophysica acta},
  year={2001},
  volume={1536 2-3},
  pages={133-40}
}
Friedreich's ataxia (FA) is a crippling neurodegenerative disease that is due to iron (Fe) overload within the mitochondrion. One therapeutic intervention may be the development of a chelator that could remove mitochondrial Fe. We have implemented the only well characterized model of mammalian mitochondrial Fe overload to examine the Fe chelation efficacy of novel chelators of the 2-pyridylcarboxaldehyde isonicotinoyl hydrazone (PCIH) class. In this model we utilize reticulocytes treated with… CONTINUE READING
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