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
M. Pandolfo, Department of Medicine, Université de
Montréal, Montréal, Québec, H2L4M1
Canada, and Department of Neurology and Neurosurgery, McGill
University, Montréal, Québec, H3A2B4
* To whom correspondence should be
Volume 276, Number 5319, Issue of 13 Jun
1997, pp. 1709-1712.
Full article is available, not free, at http://www.sciencemag.org/cgi/content/abstract/276/5319/1709
Copyright © 1997 by The American
Association for the Advancement of Science.