Abstract
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.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Animals
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COS Cells
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Frataxin
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Friedreich Ataxia / enzymology
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Friedreich Ataxia / genetics*
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Green Fluorescent Proteins
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Humans
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Introns
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Iron-Binding Proteins*
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Luminescent Proteins / biosynthesis
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Mitochondria / enzymology*
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Organelles / metabolism
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Phosphotransferases (Alcohol Group Acceptor) / biosynthesis*
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Phosphotransferases (Alcohol Group Acceptor) / genetics*
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Recombinant Fusion Proteins / biosynthesis
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Succinate Dehydrogenase / biosynthesis
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Transfection
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Trinucleotide Repeats*
Substances
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Iron-Binding Proteins
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Luminescent Proteins
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Recombinant Fusion Proteins
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Green Fluorescent Proteins
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Succinate Dehydrogenase
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Phosphotransferases (Alcohol Group Acceptor)