Frataxin gene of Friedreich's ataxia is targeted to mitochondria

Josef Priller, Clemens R. Scherzer, Peter W. Faber, Marcy E. MacDonald, Anne B. Young

Research output: Contribution to journalArticlepeer-review

91 Scopus citations


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-diquinolizino-SH-xanthylium chloride (CMXRos), a novel mitochondrial dye. Thus, frataxin appears to be a nuclear-encoded mitochondrial protein.

Original languageEnglish
Pages (from-to)265-269
Number of pages5
JournalAnnals of Neurology
Issue number2
StatePublished - Aug 1997
Externally publishedYes


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