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.
