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Science. 1997 Jun 13;276(5319):1709-12.

Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin.

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  • 1Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA.

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 in Saccharomyces 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 which YFH1 regulates iron homeostasis in yeast may help to define the pathologic process leading to cell damage in Friedreich's ataxia.

PMID:
9180083
[PubMed - indexed for MEDLINE]
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