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

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.
Authors	M Babcock, D de Silva, R Oaks, S Davis-Kaplan, S Jiralerspong, L Montermini, M Pandolfo, J Kaplan
Journal	Science (New York, N.Y.) (Science) Vol. 276 Issue 5319 Pg. 1709-12 (Jun 13 1997) ISSN: 0036-8075 [Print] United States
PMID	9180083 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References	Carrier Proteins FTR1 protein, S cerevisiae Fungal Proteins Iron-Binding Proteins Membrane Transport Proteins Recombinant Fusion Proteins Saccharomyces cerevisiae Proteins frataxin Iron Oxidoreductases Ceruloplasmin FET3 protein, S cerevisiae Phosphotransferases (Alcohol Group Acceptor)
Topics	Biological Transport Carrier Proteins (metabolism) Cell Membrane (metabolism) Ceruloplasmin Cytosol (metabolism) Friedreich Ataxia (metabolism) Fungal Proteins (genetics, metabolism) Genes, Fungal Genetic Complementation Test Homeostasis Humans Iron (metabolism) Iron-Binding Proteins Membrane Transport Proteins (metabolism) Mitochondria (metabolism) Oxidative Stress Oxidoreductases (metabolism) Phosphotransferases (Alcohol Group Acceptor) (metabolism) Recombinant Fusion Proteins (metabolism) Saccharomyces cerevisiae (genetics, growth & development, metabolism) Saccharomyces cerevisiae Proteins Transformation, Genetic

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