Frataxin gene of Friedreich's ataxia is targeted to mitochondria

J Priller, C R Scherzer, P W Faber, M E MacDonald, A B Young

Research output: Contribution to journalArticlepeer-review


Friedreich's ataxia is caused by a triplet repeat expansion in intron 1, a noncoding region of the frataxin gene (X25). We have generated a chimeric gene composed of the frataxin gene fused with the green fluorescent protein (GFP) gene as a reporter. Transfection of the fusion construct into living COS cells revealed that the frataxin-GFP construct localizes to organelles that double-label with 8-(4'-chloromethyl) phenyl-2,3,5,6,11,12,14,15-octahydro-1H,4H,10H-13H-diquinolizin o-8H-xanthylium chloride (CMXRos), a novel mitochondrial dye. Thus, frataxin appears to be a nuclear-encoded mitochondrial protein.

Original languageEnglish
Pages (from-to)265-9
Number of pages5
JournalAnnals of Neurology
Issue number2
Publication statusPublished - Aug 1997


  • Animals
  • COS Cells
  • Friedreich Ataxia
  • Green Fluorescent Proteins
  • Humans
  • Introns
  • Iron-Binding Proteins
  • Luminescent Proteins
  • Mitochondria
  • Organelles
  • Phosphotransferases (Alcohol Group Acceptor)
  • Recombinant Fusion Proteins
  • Succinate Dehydrogenase
  • Transfection
  • Trinucleotide Repeats
  • Journal Article
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

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