Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation.

  title={Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation.},
  author={Or Kakhlon and Hila Manning and William Breuer and Naomi Melamed-Book and Chunye Lu and Gino A. Cortopassi and Arnold Munnich and Zvi Ioav Cabantchik},
  volume={112 13},
Various human disorders are associated with misdistribution of iron within or across cells. Friedreich ataxia (FRDA), a deficiency in the mitochondrial iron-chaperone frataxin, results in defective use of iron and its misdistribution between mitochondria and cytosol. We assessed the possibility of functionally correcting the cellular properties affected by frataxin deficiency with a siderophore capable of relocating iron and facilitating its metabolic use. Adding the chelator deferiprone at… CONTINUE READING


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