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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 organells that double-lable with 8-(4'-chloromethyl)phenyl-2,3,5,6,11,12, 14,15-octahydro-1H,4H,10H-13H-diquinolizino-8H-xanthylium chloride (CMXRos),a novel mitochondrial dye.Thus,frataxin appears to be a nuclear- encoded mitochondrial protein. |
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