Reduced expression of frataxin extends the lifespan of Caenorhabditis elegans

Summary Defects in the expression of the mitochondrial protein frataxin cause Friedreich's ataxia, an hereditary neurodegenerative syndrome characterized by progressive ataxia and associated with reduced life expectancy in humans. Homozygous inactivation of the frataxin gene results in embryoni...

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Bibliographic Details
Published in:Aging cell 2005-04, Vol.4 (2), p.109-112
Main Authors: Ventura, Natascia, Rea, Shane, Henderson, Samuel T., Condo, Ivano, Johnson, Thomas E., Testi, Roberto
Format: Article
Language:English
Subjects:
aging
Animals
Body Size
Caenorhabditis elegans - genetics
Caenorhabditis elegans - physiology
Fertility
Frataxin
Friedreich's Ataxia
Iron-Binding Proteins - biosynthesis
Iron-Binding Proteins - genetics
Longevity
mitochondria
RNA Interference
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Summary:Summary Defects in the expression of the mitochondrial protein frataxin cause Friedreich's ataxia, an hereditary neurodegenerative syndrome characterized by progressive ataxia and associated with reduced life expectancy in humans. Homozygous inactivation of the frataxin gene results in embryonic lethality in mice, suggesting that frataxin is required for organismic survival. Intriguingly, the inactivation of many mitochondrial genes in the nematode Caenorhabditis elegans by RNAi extends lifespan. We therefore investigated whether inactivation of frataxin by RNAi‐mediated suppression of the frataxin homolog gene (frh‐1) would also prolong lifespan in the nematode. Frataxin‐deficient animals have a small body size, reduced fertility and altered responses to oxidative stress. Importantly, frataxin suppression by RNAi significantly extends lifespan in C. elegans.
ISSN:1474-9718
1474-9726
DOI:10.1111/j.1474-9726.2005.00149.x