Document Detail

Cells lacking pfh1, a fission yeast homolog of mammalian frataxin protein, display constitutive activation of the iron starvation response.
MedLine Citation:
PMID:  23115244     Owner:  NLM     Status:  MEDLINE    
Friedreich ataxia is a genetic disease caused by deficiencies in frataxin. This protein has homologs not only in higher eukaryotes but also in bacteria, fungi, and plants. The function of this protein is still controversial. We have identified a frataxin homolog in fission yeast, and we have analyzed whether its depletion leads to any of the phenotypes observed in other organisms. Cells deleted in pfh1 are sensitive to growth under aerobic conditions, display increased levels of total iron, hallmarks of oxidative stress such as protein carbonylation, decreased aconitase activity, and lower levels of oxygen consumption compared with wild-type cells. This mitochondrial protein seems to be important for iron and/or reactive oxygen species homeostasis. We have analyzed the proteome of cells devoid of Pfh1, and we determined that gene products up- and down-regulated upon iron depletion in wild-type cells are constitutively misregulated in this mutant. Because of the particular signaling pathway components governing the iron starvation response in fission yeast, our experiments suggest that cells lacking Pfh1 display a decrease of cytosolic available iron that triggers activation of Grx4, the common regulator of the iron starvation gene expression program. Our Schizosaccharomyces pombe Δpfh1 strain constitutes a new and useful model system to study Friedreich ataxia.
Natalia Gabrielli; José Ayté; Elena Hidalgo
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2012-10-31
Journal Detail:
Title:  The Journal of biological chemistry     Volume:  287     ISSN:  1083-351X     ISO Abbreviation:  J. Biol. Chem.     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-12-17     Completed Date:  2013-02-14     Revised Date:  2013-12-18    
Medline Journal Info:
Nlm Unique ID:  2985121R     Medline TA:  J Biol Chem     Country:  United States    
Other Details:
Languages:  eng     Pagination:  43042-51     Citation Subset:  IM    
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MeSH Terms
Amino Acid Sequence
DNA Helicases / chemistry,  deficiency*,  metabolism
Down-Regulation / genetics
Gene Deletion
Gene Expression Regulation, Fungal
Genes, Fungal / genetics
Iron / deficiency*
Iron-Binding Proteins / chemistry*,  metabolism
Mammals / metabolism*
Molecular Sequence Data
Proteome / metabolism
Saccharomyces cerevisiae / metabolism
Schizosaccharomyces / cytology*,  genetics,  metabolism*
Schizosaccharomyces pombe Proteins / chemistry,  genetics,  metabolism
Sequence Homology, Amino Acid*
Transcriptome / genetics
Up-Regulation / genetics
Reg. No./Substance:
0/Iron-Binding Proteins; 0/Proteome; 0/Schizosaccharomyces pombe Proteins; 0/frataxin; E1UOL152H7/Iron; EC 3.6.4.-/DNA Helicases; EC 5.99.-/Pfh1 protein, S pombe

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