Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin-Reference-Cited by-同舟云学术

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

Published:1997-06-13 Issue:5319 Volume:276 Page:1709-1712
ISSN:0036-8075
Container-title:Science
language:en
Short-container-title:Science

Author:

Babcock Michael¹²³,de Silva Deepika¹²³,Oaks Robert¹²³,Davis-Kaplan Sandra¹²³,Jiralerspong Sarn¹²³,Montermini Laura¹²³,Pandolfo Massimo¹²³,Kaplan Jerry¹²³

Affiliation:

1. 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.

2. S. Jiralerspong and L. Montermini, Centre de Recherche Louis-Charles Simard, Montréal, Québec, H2L4M1 Canada.

3. M. Pandolfo, Department of Medicine, Université de Montréal, Montréal, Québec, H2L4M1 Canada, and Department of Neurology and Neurosurgery, McGill University, Montréal, Québec, H3A2B4 Canada.

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.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

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