yeast frataxin homologue mediates mitochondrial iron efflux

Mutations in the nuclear gene encoding the mitochondrial protein frataxin are responsible for the neurological disorder Friedreich ataxia (FA). Yeast strains with a deletion in the frataxin homologue YFH1 accumulate excess iron in mitochondria and demonstrate mitochondrial damage. We show that in th...

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Bibliographic Details
Published inThe Journal of biological chemistry Vol. 274; no. 8; pp. 4497 - 4499
Main Authors Radisky, D.C, Babcock, M.C, Kaplan, J
Format Journal Article
LanguageEnglish
Published American Society for Biochemistry and Molecular Biology 01.02.1999
Subjects
active transport
cell membranes
gene expression
ion transport
iron
messenger RNA
mitochondria
proteins
Saccharomyces cerevisiae
structural genes
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Summary:Mutations in the nuclear gene encoding the mitochondrial protein frataxin are responsible for the neurological disorder Friedreich ataxia (FA). Yeast strains with a deletion in the frataxin homologue YFH1 accumulate excess iron in mitochondria and demonstrate mitochondrial damage. We show that in the absence of YFH1 , mitochondrial damage is proportional to the concentration and duration of exposure to extracellular iron, establishing mitochondrial iron accumulation as causal to mitochondrial damage. Reintroduction of YFH1 results in the rapid export of accumulated mitochondrial iron into the cytosol as free, non-heme bound iron, demonstrating that mitochondrial iron in the yeast FA model can be made bioavailable. These results demonstrate a mitochondrial iron cycle in which Yfh1p regulates mitochondrial iron efflux.
Bibliography:http://www.jbc.org/
ISSN:0021-9258
1083-351X
DOI:10.1074/jbc.274.8.4497