title: YFH1-mediated iron homeostasis is independent of mitochondrial respiration.
abstract: The human gene frataxin and its yeast homolog YFH1 affect mitochondrial function. Deficits in frataxin result in Friedreich ataxia, while deletion of YFH1 results in respiratory incompetence. We determined that as long as respiratory incompetent yeast express Yfh1p they do not accumulate excessive mitochondrial iron. Deletion of YFH1 in respiratory incompetent yeast results in mitochondrial iron accumulation, while the reintroduction of Yfh1p results in mitochondrial iron export. Further, overexpression of Yfh1p has no effect on oxygen consumption in wild-type yeast grown in either fermentative or respiratory carbon sources. We conclude that the effect of Yfh1p on mitochondrial iron metabolism is independent of respiratory activity.
authors.affiliation: Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA.
authors.forename: O S
authors.initials: OS
authors.lastname: Chen
authors.forename: J
authors.initials: J
authors.lastname: Kaplan
mesh_terms: D004272:DNA, Mitochondrial / Q000378:metabolism; D004791:Enzyme Inhibitors / Q000494:pharmacology; D004996:Ethidium / Q000494:pharmacology; D005456:Fluorescent Dyes / Q000494:pharmacology; D017353:Gene Deletion; D007501:Iron / Q000378:metabolism*; D033862:Iron-Binding Proteins; D008928:Mitochondria / Q000378:metabolism*; D010100:Oxygen / Q000378:metabolism; D010101:Oxygen Consumption; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000378:metabolism* / Q000502:physiology*; D010957:Plasmids / Q000378:metabolism; D012441:Saccharomyces cerevisiae / Q000378:metabolism*; D013347:Subcellular Fractions; D000096923:Frataxin
chemical_list: D004272:DNA, Mitochondrial; D004791:Enzyme Inhibitors; D004996:Ethidium; D005456:Fluorescent Dyes; D007501:Iron; D033862:Iron-Binding Proteins; D010100:Oxygen; D017853:Phosphotransferases (Alcohol Group Acceptor); D000096923:Frataxin