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

 Michael Babcock, Deepika de Silva, Robert Oaks, Sandra Davis-Kaplan, Sarn Jiralerspong, Laura Montermini, Massimo Pandolfo, Jerry Kaplan *

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.

M. Babcock, D. de Silva, R. Oaks, S. Davis-Kaplan, J. Kaplan, Division of Immunology and Cell Biology, Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA.
S. Jiralerspong and L. Montermini, Centre de Recherche Louis-Charles Simard, Montréal, Québec, H2L4M1 Canada.
M. Pandolfo, Department of Medicine, Université de Montréal, Montréal, Québec, H2L4M1 Canada, and Department of Neurology and Neurosurgery, McGill University, Montréal, Québec, H3A2B4 Canada.

* To whom correspondence should be addressed.

Volume 276, Number 5319, Issue of 13 Jun 1997, pp. 1709-1712.
Full article is available, not free, at

Copyright © 1997 by The American Association for the Advancement of Science.