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.
| Original language | English |
|---|---|
| Pages (from-to) | 265-9 |
| Number of pages | 5 |
| Journal | Annals of Neurology |
| Volume | 42 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - Aug 1997 |
Keywords / Materials (for Non-textual outputs)
- 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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