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Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin

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Science  13 Jun 1997:
Vol. 276, Issue 5319, pp. 1709-1712
DOI: 10.1126/science.276.5319.1709

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Abstract

The gene responsible for Friedreich's ataxia, a disease characterized by neurodegeneration and cardiomyopathy, has recently been cloned and its product designated frataxin. A gene inSaccharomyces cerevisiae was characterized whose predicted protein product has high sequence similarity to the human frataxin protein. The yeast gene (yeast frataxin homolog, YFH1) encodes a mitochondrial protein involved in iron homeostasis and respiratory function. Human frataxin also was shown to be a mitochondrial protein. Characterizing the mechanism by whichYFH1 regulates iron homeostasis in yeast may help to define the pathologic process leading to cell damage in Friedreich's ataxia.

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