Frataxin gene of Friedreich's ataxia is targeted to mitochondria
Annals of NeurologyPublished 1 August 1997
Josef Priller, Clemens R. Scherzer, Peter W. Faber, Marcy E. MacDonald, Anne B. Yong
Citations100
SJR quartileQ1
SJR score3.74
SNIP2.26
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TL;DR
It is 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‐8H‐xanthylium chloride (CMXRos), a novel mitochondrial dye.
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.
Keywords
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